Raras
Buscar doenças, sintomas, genes...
Alteração do metabolismo da melanina
ORPHA:352728DOENÇA RARA

Acne é uma condição cutânea de longa duração caracterizada por áreas de pontos negros, pontos brancos, pústulas, pele oleosa e possibilidade de aparecimento de cicatrizes. Dependendo do grau de incidência, infecção e dimensão dos pontos de acne sobre a pele, as consequências na aparência podem provocar desde desconfortos pontuais, passando por ansiedade, diminuição da autoestima até, em casos extremos, depressão e pensamentos de suicídio.

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Introdução

O que você precisa saber de cara

📋

Doença rara caracterizada por defeitos na produção e distribuição de melanina, associada a manifestações neurológicas (crises tônico-clônicas, neuropatia), imunológicas (infecções recorrentes) e morfológicas (polegar anormal, calcificações cerebrais).

Medicamentos
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SUS: Cobertura mínimaScore: 20%
Centros em: PA, PR, SC, RS, ES +8
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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

👁️
Olhos
40 sintomas
🧠
Neurológico
33 sintomas
🧬
Pele e cabelo
29 sintomas
🩸
Sangue
20 sintomas
🫃
Digestivo
14 sintomas
😀
Face
12 sintomas

+ 115 sintomas em outras categorias

Características mais comuns

Morfologia anormal do polegar
Crise tônico-clônica bilateral
Infecções recorrentes do trato respiratório superior
Calcificação cerebral
Agregação plaquetária prejudicada
Agregação plaquetária induzida por colágeno prejudicada
308sintomas
Sem dados (308)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 308 características clínicas mais associadas, ordenadas por frequência.

Morfologia anormal do polegarAbnormal thumb morphology
Crise tônico-clônica bilateralBilateral tonic-clonic seizure
Infecções recorrentes do trato respiratório superiorRecurrent upper respiratory tract infections
Calcificação cerebralCerebral calcification
Agregação plaquetária prejudicadaImpaired platelet aggregation

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1
Últimos 10 anos200publicações
Pico2025143 papers
Linha do tempo
2025Hoje · 2026🧪 2013Primeiro ensaio clínico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

24 genes identificados com associação a esta condição.

TYRTyrosinaseDisease-causing germline mutation(s) inTolerante
FUNÇÃO

This is a copper-containing oxidase that functions in the formation of pigments such as melanins and other polyphenolic compounds. Catalyzes the initial and rate limiting step in the cascade of reactions leading to melanin production from tyrosine (By similarity). In addition to hydroxylating tyrosine to DOPA (3,4-dihydroxyphenylalanine), also catalyzes the oxidation of DOPA to DOPA-quinone, and possibly the oxidation of DHI (5,6-dihydroxyindole) to indole-5,6 quinone (PubMed:28661582)

LOCALIZAÇÃO

Melanosome membraneMelanosome

VIAS BIOLÓGICAS (2)
Melanin biosynthesisRegulation of MITF-M-dependent genes involved in pigmentation
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 1A

An autosomal recessive disorder in which the biosynthesis of melanin pigment is absent in skin, hair, and eyes. It is characterized by complete lack of tyrosinase activity due to production of an inactive enzyme. Patients present with a life-long absence of melanin pigment after birth, and manifest increased sensitivity to ultraviolet radiation with predisposition to skin cancer. Visual anomalies include decreased acuity, nystagmus, strabismus and photophobia.

EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Sun Exposed Lower leg
7.7 TPM
Skin Not Sun Exposed Suprapubic
6.6 TPM
Aorta
0.1 TPM
Testículo
0.1 TPM
Glândula salivar
0.1 TPM
OUTRAS DOENÇAS (5)
oculocutaneous albinism type 1Boculocutaneous albinism type 1Aminimal pigment oculocutaneous albinism type 1Waardenburg syndrome type 2
HGNC:12442UniProt:P14679
HPS6BLOC-2 complex member HPS6Disease-causing germline mutation(s) inTolerante
FUNÇÃO

May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules (PubMed:17041891). Acts as a cargo adapter for the dynein-dynactin motor complex to mediate the transport of lysosomes from the cell periphery to the perinuclear region. Facilitates retrograde lysosomal trafficking by linking the motor complex to lysosomes, and perinuclear positioning of lysosomes is crucial for the delivery of endocytic cargos to lysosomes,

LOCALIZAÇÃO

Microsome membraneCytoplasm, cytosolEarly endosome membraneLysosome membrane

MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 6

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
30.4 TPM
Linfócitos
26.4 TPM
Fallopian Tube
19.4 TPM
Útero
18.1 TPM
Baço
17.0 TPM
OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 6Hermansky-Pudlak syndrome without pulmonary fibrosis
HGNC:18817UniProt:Q86YV9
DCTL-dopachrome tautomeraseDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Plays a role in melanin biosynthesis (PubMed:33100333). Catalyzes the conversion of L-dopachrome into 5,6-dihydroxyindole-2-carboxylic acid (DHICA)

LOCALIZAÇÃO

Melanosome membraneMelanosome

VIAS BIOLÓGICAS (2)
Melanin biosynthesisRegulation of MITF-M-dependent genes involved in pigmentation
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 8

A form of oculocutaneous albinism, a disorder of pigmentation characterized by reduced biosynthesis of melanin in the skin, hair and eyes. OCA8 is an autosomal recessive form characterized by mild hair and skin hypopigmentation, associated with ocular features including nystagmus, reduced visual acuity, iris transillumination, and hypopigmentation of the retina.

EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Sun Exposed Lower leg
24.2 TPM
Skin Not Sun Exposed Suprapubic
23.2 TPM
Pituitária
3.0 TPM
Testículo
1.8 TPM
Brain Nucleus accumbens basal ganglia
1.6 TPM
OUTRAS DOENÇAS (1)
oculocutaneous albinism type 8
HGNC:2709UniProt:P40126
SLC45A2Membrane-associated transporter proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Proton-associated glucose and sucrose transporter (By similarity). May be able to transport also fructose (By similarity). Expressed at a late melanosome maturation stage where functions as proton/glucose exporter which increase lumenal pH by decreasing glycolysis (PubMed:32966160, PubMed:35469906). Regulates melanogenesis by maintaining melanosome neutralization that is initially initiated by transient OCA2 and required for a proper function of the tyrosinase TYR (PubMed:32966160, PubMed:354699

LOCALIZAÇÃO

Melanosome membrane

VIAS BIOLÓGICAS (1)
Melanin biosynthesis
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 4

A disorder of pigmentation characterized by reduced biosynthesis of melanin in the skin, hair and eyes. Patients show reduced or lacking pigmentation associated with classic albinism ocular abnormalities, including decreased visual acuity, macular hypoplasia, optic dysplasia, atypical choroidal vessels, and nystagmus.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Baixa expressão)
Testículo
1.6 TPM
Skin Sun Exposed Lower leg
0.5 TPM
Rim - Medula
0.4 TPM
Skin Not Sun Exposed Suprapubic
0.4 TPM
Fígado
0.3 TPM
OUTRAS DOENÇAS (2)
obsolete skin/hair/eye pigmentation, variation in, 5oculocutaneous albinism type 4
HGNC:16472UniProt:Q9UMX9
MLPHMelanophilinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Rab effector protein involved in melanosome transport. Serves as link between melanosome-bound RAB27A and the motor protein MYO5A

LOCALIZAÇÃO

Cytoplasm

VIAS BIOLÓGICAS (1)
Regulation of MITF-M-dependent genes involved in pigmentation
MECANISMO DE DOENÇA

Griscelli syndrome 3

Rare autosomal recessive disorder characterized by pigmentary dilution of the skin and hair, the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes, without other clinical manifestations.

EXPRESSÃO TECIDUAL(Ubíquo)
Próstata
139.8 TPM
Glândula salivar
132.9 TPM
Cervix Ectocervix
114.9 TPM
Cervix Endocervix
82.7 TPM
Estômago
72.1 TPM
OUTRAS DOENÇAS (1)
Griscelli syndrome type 3
HGNC:29643UniProt:Q9BV36
HPS3BLOC-2 complex member HPS3Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Involved in early stages of melanosome biogenesis and maturation

LOCALIZAÇÃO

CytoplasmCytoplasm, cytosol

MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 3

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
27.4 TPM
Linfócitos
24.4 TPM
Útero
22.8 TPM
Nervo tibial
21.4 TPM
Baço
18.9 TPM
OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 3Hermansky-Pudlak syndrome without pulmonary fibrosis
HGNC:15597UniProt:Q969F9
DTNBP1DysbindinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes. In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. Associates with the BLOC-2 complex

LOCALIZAÇÃO

CytoplasmCytoplasmic vesicle membraneEndosome membraneMelanosome membranePostsynaptic densityEndoplasmic reticulumNucleusCytoplasmic vesicle, secretory vesicle, synaptic vesicle membranePostsynaptic cell membrane

VIAS BIOLÓGICAS (1)
Golgi Associated Vesicle Biogenesis
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 7

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Brain Nucleus accumbens basal ganglia
37.2 TPM
Artéria tibial
29.7 TPM
Aorta
25.4 TPM
Cólon sigmoide
24.0 TPM
Brain Frontal Cortex BA9
21.5 TPM
OUTRAS DOENÇAS (1)
Hermansky-Pudlak syndrome 7
HGNC:17328UniProt:Q96EV8
SLC24A5Sodium/potassium/calcium exchanger 5Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Calcium, potassium:sodium antiporter that transports 1 Ca(2+) and 1 K(+) to the melanosome in exchange for 4 cytoplasmic Na(+) (PubMed:18166528). Involved in pigmentation, possibly by participating in ion transport in melanosomes (PubMed:16357253, PubMed:18166528). Predominant sodium-calcium exchanger in melanocytes (PubMed:16357253, PubMed:18166528)

LOCALIZAÇÃO

Golgi apparatus, trans-Golgi network membraneMelanosome

VIAS BIOLÓGICAS (1)
Sodium/Calcium exchangers
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 6

A disorder characterized by a reduction or complete loss of melanin in the skin, hair and eyes. Patients show reduced or lacking pigmentation often accompanied by eye symptoms such as photophobia, strabismus, moderate to severe visual impairment, and nystagmus.

EXPRESSÃO TECIDUAL(Não detectado)
Skin Not Sun Exposed Suprapubic
0.3 TPM
Skin Sun Exposed Lower leg
0.3 TPM
Testículo
0.2 TPM
Pituitária
0.1 TPM
Cérebro - Hemisfério cerebelar
0.1 TPM
OUTRAS DOENÇAS (1)
oculocutaneous albinism type 6
HGNC:20611UniProt:Q71RS6
AP3B1AP-3 complex subunit beta-1Disease-causing germline mutation(s) inRestrito
FUNÇÃO

Subunit of non-clathrin- and clathrin-associated adaptor protein complex 3 (AP-3) that plays a role in protein sorting in the late-Golgi/trans-Golgi network (TGN) and/or endosomes. The AP complexes mediate both the recruitment of clathrin to membranes and the recognition of sorting signals within the cytosolic tails of transmembrane cargo molecules. AP-3 appears to be involved in the sorting of a subset of transmembrane proteins targeted to lysosomes and lysosome-related organelles. In concert w

LOCALIZAÇÃO

Cytoplasmic vesicle, clathrin-coated vesicle membraneGolgi apparatus

VIAS BIOLÓGICAS (1)
Golgi Associated Vesicle Biogenesis
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 2

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. HPS2 differs from the other forms of HPS in that it includes immunodeficiency in its phenotype and patients with HPS2 have an increased susceptibility to infections.

OUTRAS DOENÇAS (1)
Hermansky-Pudlak syndrome 2
HGNC:566UniProt:O00203
LYSTLysosomal-trafficking regulatorDisease-causing germline mutation(s) inRestrito
FUNÇÃO

Adapter protein that regulates and/or fission of intracellular vesicles such as lysosomes (PubMed:11984006, PubMed:25216107). Might regulate trafficking of effectors involved in exocytosis (PubMed:25425525). In cytotoxic T-cells and natural killer (NK) cells, has role in the regulation of size, number and exocytosis of lytic granules (PubMed:26478006). In macrophages and dendritic cells, regulates phagosome maturation by controlling the conversion of early phagosomal compartments into late phago

LOCALIZAÇÃO

Cytoplasm

VIAS BIOLÓGICAS (3)
RUNX2 regulates osteoblast differentiationFormation of the anterior neural plateFormation of the posterior neural plate
MECANISMO DE DOENÇA

Chediak-Higashi syndrome

A rare autosomal recessive disorder characterized by hypopigmentation, severe immunologic deficiency, a bleeding tendency, neurologic abnormalities, abnormal intracellular transport to and from the lysosome, and giant inclusion bodies in a variety of cell types. Most patients die at an early age unless they receive an allogeneic hematopoietic stem cell transplant (SCT).

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
14.6 TPM
Cerebelo
14.6 TPM
Nervo tibial
12.8 TPM
Tecido adiposo
12.7 TPM
Skin Not Sun Exposed Suprapubic
12.2 TPM
OUTRAS DOENÇAS (2)
Chediak-Higashi syndromeattenuated Chédiak-Higashi syndrome
HGNC:1968UniProt:Q99698
GPR143G-protein coupled receptor 143Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Receptor for tyrosine, L-DOPA and dopamine. After binding to L-DOPA, stimulates Ca(2+) influx into the cytoplasm, increases secretion of the neurotrophic factor SERPINF1 and relocalizes beta arrestin at the plasma membrane; this ligand-dependent signaling occurs through a G(q)-mediated pathway in melanocytic cells. Its activity is mediated by G proteins which activate the phosphoinositide signaling pathway. Also plays a role as an intracellular G protein-coupled receptor involved in melanosome b

LOCALIZAÇÃO

Melanosome membraneLysosome membraneApical cell membrane

VIAS BIOLÓGICAS (3)
G alpha (q) signalling eventsAmine ligand-binding receptorsRegulation of MITF-M-dependent genes involved in pigmentation
MECANISMO DE DOENÇA

Albinism ocular 1

Form of albinism affecting only the eye. Pigment of the hair and skin is normal or only slightly diluted. Eyes may be severely affected with photophobia and reduced visual acuity. Nystagmus or strabismus are often associated. The irides and fundus are depigmented.

EXPRESSÃO TECIDUAL(Ubíquo)
Brain Caudate basal ganglia
6.5 TPM
Brain Nucleus accumbens basal ganglia
6.5 TPM
Brain Putamen basal ganglia
5.5 TPM
Brain Anterior cingulate cortex BA24
5.2 TPM
Córtex cerebral
5.1 TPM
OUTRAS DOENÇAS (2)
X-linked recessive ocular albinismnystagmus 6, congenital, X-linked
HGNC:20145UniProt:P51810
AP3D1AP-3 complex subunit delta-1Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Part of the AP-3 complex, an adaptor-related complex which is not clathrin-associated. The complex is associated with the Golgi region as well as more peripheral structures. It facilitates the budding of vesicles from the Golgi membrane and may be directly involved in trafficking to lysosomes. Involved in process of CD8+ T-cell and NK cell degranulation (PubMed:26744459). In concert with the BLOC-1 complex, AP-3 is required to target cargos into vesicles assembled at cell bodies for delivery int

LOCALIZAÇÃO

CytoplasmGolgi apparatus membrane

MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 10

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. HPS10 patients manifest albinism, neutropenia, immunodeficiency, neurodevelopmental delay, generalized seizures, and impaired hearing.

OUTRAS DOENÇAS (3)
Hermansky-Pudlak syndrome 10ocular albinism with late-onset sensorineural deafnessX-linked recessive ocular albinism
HGNC:568UniProt:O14617
MC1RMelanocyte-stimulating hormone receptorCandidate gene tested inDesconhecido
FUNÇÃO

G protein-coupled receptor that binds melanocyte-stimulating hormones (alpha, beta, and gamma-MSH) and adrenocorticotropic hormone/ACTH, which are peptide products of the POMC precursor protein (PubMed:11442765, PubMed:11707265, PubMed:1325670, PubMed:1516719, PubMed:8463333). Upon activation, MC1R couples with the G(s) protein, stimulating adenylate cyclase and activating the cAMP-dependent signaling pathway. This activation promotes melanogenesis, resulting in the production of eumelanin (blac

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (3)
G alpha (s) signalling eventsPeptide ligand-binding receptorsTranscriptional and post-translational regulation of MITF-M expression and activity
MECANISMO DE DOENÇA

Melanoma, cutaneous malignant 5

A malignant neoplasm of melanocytes, arising de novo or from a pre-existing benign nevus, which occurs most often in the skin but may also involve other sites.

EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
53.9 TPM
Pituitária
34.8 TPM
Cerebelo
32.6 TPM
Cérebro - Hemisfério cerebelar
29.1 TPM
Tireoide
26.9 TPM
OUTRAS DOENÇAS (4)
obsolete skin/hair/eye pigmentation, variation in, 2familial melanomaoculocutaneous albinism type 2melanoma, cutaneous malignant, susceptibility to, 5
HGNC:6929UniProt:Q01726
MYO5AUnconventional myosin-VaDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Processive actin-based motor that can move in large steps approximating the 36-nm pseudo-repeat of the actin filament. Can hydrolyze ATP in the presence of actin, which is essential for its function as a motor protein (PubMed:10448864). Involved in melanosome transport. Also mediates the transport of vesicles to the plasma membrane (By similarity). May also be required for some polarization process involved in dendrite formation (By similarity)

LOCALIZAÇÃO

VIAS BIOLÓGICAS (5)
Regulation of actin dynamics for phagocytic cup formationFCGR3A-mediated phagocytosisTranslocation of SLC2A4 (GLUT4) to the plasma membraneRegulation of MITF-M-dependent genes involved in pigmentationInsulin processing
MECANISMO DE DOENÇA

Griscelli syndrome 1

Rare autosomal recessive disorder that results in pigmentary dilution of the skin and hair, the presence of large clumps of pigment in hair shafts, silvery-gray hair and accumulation of melanosomes in melanocytes. GS1 patients show developmental delay, hypotonia and intellectual disability, without apparent immune abnormalities.

EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
68.3 TPM
Cerebelo
59.0 TPM
Brain Frontal Cortex BA9
53.8 TPM
Córtex cerebral
36.2 TPM
Brain Anterior cingulate cortex BA24
33.8 TPM
OUTRAS DOENÇAS (3)
Griscelli syndrome type 1Griscelli syndrome type 3neuroectodermal melanolysosomal disease
HGNC:7602UniProt:Q9Y4I1
BLOC1S5Biogenesis of lysosome-related organelles complex 1 subunit 5Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes (PubMed:32565547). In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. Plays a role in i

LOCALIZAÇÃO

MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 11

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 11Hermansky-Pudlak syndrome 7
HGNC:18561UniProt:Q8TDH9
TYRP15,6-dihydroxyindole-2-carboxylic acid oxidaseDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Plays a role in melanin biosynthesis (PubMed:16704458, PubMed:22556244, PubMed:23504663). Catalyzes the oxidation of 5,6-dihydroxyindole-2-carboxylic acid (DHICA) into indole-5,6-quinone-2-carboxylic acid in the presence of bound Cu(2+) ions, but not in the presence of Zn(2+) (PubMed:28661582). May regulate or influence the type of melanin synthesized (PubMed:16704458, PubMed:22556244). Also to a lower extent, capable of hydroxylating tyrosine and producing melanin (By similarity)

LOCALIZAÇÃO

Melanosome membrane

VIAS BIOLÓGICAS (2)
Melanin biosynthesisRegulation of MITF-M-dependent genes involved in pigmentation
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 3

An autosomal recessive disorder in which the biosynthesis of melanin pigment is reduced in skin, hair, and eyes. Tyrosinase activity is normal and patients have only moderate reduction of pigment. The eyes present red reflex on transillumination of the iris, dilution of color of iris, nystagmus and strabismus. Darker-skinned individuals have bright copper-red coloration of the skin and hair.

EXPRESSÃO TECIDUAL(Tecido-específico)
Skin Sun Exposed Lower leg
40.8 TPM
Skin Not Sun Exposed Suprapubic
30.8 TPM
Coração - Ventrículo esquerdo
9.8 TPM
Coração - Átrio
6.8 TPM
Rim - Medula
4.8 TPM
OUTRAS DOENÇAS (2)
obsolete skin/hair/eye pigmentation, variation in, 11oculocutaneous albinism type 3
HGNC:12450UniProt:P17643
HPS4BLOC-3 complex member HPS4Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-3 complex, a complex that acts as a guanine exchange factor (GEF) for RAB32 and RAB38, promotes the exchange of GDP to GTP, converting them from an inactive GDP-bound form into an active GTP-bound form. The BLOC-3 complex plays an important role in the control of melanin production and melanosome biogenesis and promotes the membrane localization of RAB32 and RAB38 (PubMed:23084991)

LOCALIZAÇÃO

VIAS BIOLÓGICAS (1)
RAB GEFs exchange GTP for GDP on RABs
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 4

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Cerebelo
67.3 TPM
Cérebro - Hemisfério cerebelar
66.6 TPM
Testículo
44.7 TPM
Skin Sun Exposed Lower leg
40.8 TPM
Skin Not Sun Exposed Suprapubic
36.3 TPM
OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 4Hermansky-Pudlak syndrome with pulmonary fibrosis
HGNC:15844UniProt:Q9NQG7
HPS1BLOC-3 complex member HPS1Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-3 complex, a complex that acts as a guanine exchange factor (GEF) for RAB32 and RAB38, promotes the exchange of GDP to GTP, converting them from an inactive GDP-bound form into an active GTP-bound form. The BLOC-3 complex plays an important role in the control of melanin production and melanosome biogenesis and promotes the membrane localization of RAB32 and RAB38 (PubMed:23084991)

LOCALIZAÇÃO

VIAS BIOLÓGICAS (1)
RAB GEFs exchange GTP for GDP on RABs
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 1

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Baço
57.7 TPM
Cervix Endocervix
54.4 TPM
Cervix Ectocervix
48.5 TPM
Sangue
48.2 TPM
Útero
47.2 TPM
OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 1Hermansky-Pudlak syndrome with pulmonary fibrosis
HGNC:5163UniProt:Q92902
BLOC1S6Biogenesis of lysosome-related organelles complex 1 subunit 6Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes. In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. May play a role in intracellular ve

LOCALIZAÇÃO

CytoplasmMembrane

VIAS BIOLÓGICAS (1)
Golgi Associated Vesicle Biogenesis
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 9

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 9Hermansky-Pudlak syndrome 7
HGNC:8549UniProt:Q9UL45
RAB27ARas-related protein Rab-27ADisease-causing germline mutation(s) inTolerante
FUNÇÃO

The small GTPases Rab are key regulators of intracellular membrane trafficking, from the formation of transport vesicles to their fusion with membranes. Rabs cycle between an inactive GDP-bound form and an active GTP-bound form that is able to recruit to membranes different sets of downstream effectors directly responsible for vesicle formation, movement, tethering and fusion (PubMed:30771381). RAB27A regulates homeostasis of late endocytic pathway, including endosomal positioning, maturation an

LOCALIZAÇÃO

MembraneMelanosomeLate endosomeLysosome

VIAS BIOLÓGICAS (2)
RAB geranylgeranylationInsulin processing
MECANISMO DE DOENÇA

Griscelli syndrome 2

Rare autosomal recessive disorder that results in pigmentary dilution of the skin and hair, the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. GS2 patients also develop an uncontrolled T-lymphocyte and macrophage activation syndrome, known as hemophagocytic syndrome, leading to death in the absence of bone marrow transplantation. Neurological impairment is present in some patients, likely as a result of hemophagocytic syndrome.

EXPRESSÃO TECIDUAL(Ubíquo)
Sangue
45.3 TPM
Pituitária
44.8 TPM
Pulmão
38.4 TPM
Próstata
31.2 TPM
Aorta
29.4 TPM
OUTRAS DOENÇAS (1)
Griscelli syndrome type 2
HGNC:9766UniProt:P51159
OCA2P proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Contributes to a melanosome-specific anion (chloride) current that modulates melanosomal pH for optimal tyrosinase activity required for melanogenesis and the melanosome maturation (PubMed:11310796, PubMed:15262401, PubMed:22234890, PubMed:25513726). One of the components of the mammalian pigmentary system (PubMed:15262401, PubMed:18252222, PubMed:7601462). May serve as a key control point at which ethnic skin color variation is determined. Major determinant of brown and/or blue eye color (PubMe

LOCALIZAÇÃO

Melanosome membrane

VIAS BIOLÓGICAS (1)
Melanin biosynthesis
MECANISMO DE DOENÇA

Albinism, oculocutaneous, 2

An autosomal recessive disorder in which the biosynthesis of melanin pigment is reduced in skin, hair, and eyes. Although affected infants may appear at birth to have complete absence of melanin pigment, most patients acquire small amounts of pigment with age. Visual anomalies include decreased acuity and nystagmus. The phenotype is highly variable. The hair of affected individuals may turn darker with age, and pigmented nevi or freckles may be seen. African and African American individuals may have yellow hair and blue-gray or hazel irides. One phenotypic variant, 'brown OCA,' has been described in African and African American populations and is characterized by light brown hair and skin color and gray to tan irides.

VIAS REACTOME (1)
EXPRESSÃO TECIDUAL(Tecido-específico)
Artéria tibial
10.5 TPM
Tireoide
9.2 TPM
Aorta
8.2 TPM
Skin Sun Exposed Lower leg
7.1 TPM
Testículo
5.3 TPM
OUTRAS DOENÇAS (6)
obsolete skin/hair/eye pigmentation, variation in, 1oculocutaneous albinism type 2Angelman syndrome due to maternal 15q11q13 deletionPrader-Willi syndrome due to maternal uniparental disomy of chromosome 15
HGNC:8101UniProt:Q04671
BLOC1S3Biogenesis of lysosome-related organelles complex 1 subunit 3Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Component of the BLOC-1 complex, a complex that is required for normal biogenesis of lysosome-related organelles (LRO), such as platelet dense granules and melanosomes. In concert with the AP-3 complex, the BLOC-1 complex is required to target membrane protein cargos into vesicles assembled at cell bodies for delivery into neurites and nerve terminals. The BLOC-1 complex, in association with SNARE proteins, is also proposed to be involved in neurite extension. Plays a role in intracellular vesic

LOCALIZAÇÃO

Cytoplasm

VIAS BIOLÓGICAS (1)
Golgi Associated Vesicle Biogenesis
MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 8

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 8Hermansky-Pudlak syndrome 7
HGNC:20914UniProt:Q6QNY0
LRMDALeucine-rich melanocyte differentiation-associated proteinDisease-causing germline mutation(s) inTolerante
FUNÇÃO

Required for melanocyte differentiation

LOCALIZAÇÃO

MECANISMO DE DOENÇA

Albinism, oculocutaneous, 7

A disorder of pigmentation characterized by reduced biosynthesis of melanin in the skin, hair and eyes. Patients show reduced or lacking pigmentation associated with classic albinism ocular abnormalities, including decreased visual acuity, macular hypoplasia, optic dysplasia, atypical choroidal vessels, and nystagmus.

OUTRAS DOENÇAS (1)
oculocutaneous albinism type 7
HGNC:23405UniProt:Q9H2I8
HPS5BLOC-2 complex member HPS5Disease-causing germline mutation(s) inTolerante
FUNÇÃO

May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Regulates intracellular vesicular trafficking in fibroblasts. May be involved in the regulation of general functions of integrins

LOCALIZAÇÃO

Cytoplasm, cytosol

MECANISMO DE DOENÇA

Hermansky-Pudlak syndrome 5

A form of Hermansky-Pudlak syndrome, a genetically heterogeneous autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS.

EXPRESSÃO TECIDUAL(Ubíquo)
Nervo tibial
30.6 TPM
Testículo
25.4 TPM
Linfócitos
25.0 TPM
Fibroblastos
23.6 TPM
Aorta
20.7 TPM
OUTRAS DOENÇAS (2)
Hermansky-Pudlak syndrome 5Hermansky-Pudlak syndrome without pulmonary fibrosis
HGNC:17022UniProt:Q9UPZ3

Medicamentos e terapias

CARBIDOPA ANHYDROUSPhase 2

Mecanismo: DOPA decarboxylase inhibitor

SORAFENIBPhase 2

Mecanismo: Vascular endothelial growth factor receptor inhibitor

BOSENTANPhase 2

Mecanismo: Endothelin receptor, ET-A/ET-B antagonist

LEVODOPAPhase 2

Mecanismo: Dopamine D3 receptor agonist

CARBIDOPAPhase 2

Mecanismo: DOPA decarboxylase inhibitor

LETROZOLEPhase 2

Mecanismo: Cytochrome P450 19A1 inhibitor

PENTOXIFYLLINEPhase 1

Mecanismo: 3',5'-cyclic phosphodiesterase inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

695 variantes patogênicas registradas no ClinVar.

🧬 TYR: NM_000372.5(TYR):c.158G>A (p.Gly53Asp) ()
🧬 TYR: NM_000372.5(TYR):c.308G>C (p.Cys103Ser) ()
🧬 TYR: NM_000372.5(TYR):c.411C>A (p.Tyr137Ter) ()
🧬 TYR: NM_000372.5(TYR):c.1147G>C (p.Asp383His) ()
🧬 TYR: NM_000372.5(TYR):c.866G>C (p.Cys289Ser) ()
Ver todas no ClinVar

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Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Alteração do metabolismo da melanina

Centros de Referência SUS

21 centros habilitados pelo SUS para Alteração do metabolismo da melanina

Centros para Alteração do metabolismo da melanina

Detalhes dos centros

Hospital Universitário Prof. Edgard Santos (HUPES)

R. Dr. Augusto Viana, s/n - Canela, Salvador - BA, 40110-060 · CNES 0003808

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital de Apoio de Brasília (HAB)

AENW 3 Lote A Setor Noroeste - Plano Piloto, Brasília - DF, 70684-831 · CNES 0010456

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Estadual Infantil e Maternidade Alzir Bernardino Alves (HIABA)

Av. Min. Salgado Filho, 918 - Soteco, Vila Velha - ES, 29106-010 · CNES 6631207

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Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital das Clínicas da UFG

Rua 235 QD. 68 Lote Área, Nº 285, s/nº - Setor Leste Universitário, Goiânia - GO, 74605-050 · CNES 2338424

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da UFMG

Av. Prof. Alfredo Balena, 110 - Santa Efigênia, Belo Horizonte - MG, 30130-100 · CNES 2280167

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

NUPAD / Faculdade de Medicina UFMG

Av. Prof. Alfredo Balena, 189 - 5 andar - Centro, Belo Horizonte - MG, 30130-100 · CNES 2183226

Serviço de Referência

Rota
Erros Inatos do Metabolismo

Hospital Universitário João de Barros Barreto

R. dos Mundurucus, 4487 - Guamá, Belém - PA, 66073-000 · CNES 2337878

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Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas da Universidade Federal de Pernambuco

Av. Prof. Moraes Rego, 1235 - Cidade Universitária, Recife - PE, 50670-901 · CNES 2561492

Atenção Especializada

Rota
Erros Inatos do Metabolismo

Instituto de Medicina Integral Prof. Fernando Figueira (IMIP)

R. dos Coelhos, 300 - Boa Vista, Recife - PE, 50070-902 · CNES 0000647

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas da UFPR

R. Gen. Carneiro, 181 - Alto da Glória, Curitiba - PR, 80060-900 · CNES 2364980

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Pedro Ernesto (HUPE-UERJ)

Blvd. 28 de Setembro, 77 - Vila Isabel, Rio de Janeiro - RJ, 20551-030 · CNES 2280221

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Instituto Nacional de Saúde da Mulher, da Criança e do Adolescente Fernandes Figueira (IFF/Fiocruz)

Av. Rui Barbosa, 716 - Flamengo, Rio de Janeiro - RJ, 22250-020 · CNES 2269988

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Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário Onofre Lopes (HUOL)

Av. Nilo Peçanha, 620 - Petrópolis, Natal - RN, 59012-300 · CNES 2408570

Atenção Especializada

Rota
Erros Inatos do Metabolismo

Hospital São Lucas da PUCRS

Av. Ipiranga, 6690 - Jardim Botânico, Porto Alegre - RS, 90610-000 · CNES 2232928

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Anomalias CongênitasErros Inatos do Metabolismo

Hospital de Clínicas de Porto Alegre (HCPA)

Rua Ramiro Barcelos, 2350 Bloco A - Av. Protásio Alves, 211 - Bloco B e C - Santa Cecília, Porto Alegre - RS, 90035-903 · CNES 2237601

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Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital Universitário da UFSC (HU-UFSC)

R. Profa. Maria Flora Pausewang - Trindade, Florianópolis - SC, 88036-800 · CNES 2560356

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do Metabolismo

Hospital das Clínicas da FMUSP

R. Dr. Ovídio Pires de Campos, 225 - Cerqueira César, São Paulo - SP, 05403-010 · CNES 2077485

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas da UNICAMP

R. Vital Brasil, 251 - Cidade Universitária, Campinas - SP, 13083-888 · CNES 2748223

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Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Hospital de Clínicas de Ribeirão Preto (HCRP-USP)

R. Ten. Catão Roxo, 3900 - Vila Monte Alegre, Ribeirão Preto - SP, 14015-010 · CNES 2082187

Serviço de Referência

Rota
Anomalias CongênitasErros Inatos do MetabolismoDeficiência Intelectual

Instituto da Criança e do Adolescente (ICr-HCFMUSP)

Av. Dr. Enéas Carvalho de Aguiar, 647 - Cerqueira César, São Paulo - SP, 05403-000 · CNES 2081695

Serviço de Referência

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UNIFESP / Hospital São Paulo

R. Napoleão de Barros, 715 - Vila Clementino, São Paulo - SP, 04024-002 · CNES 2688689

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Sobre os centros SUS: Estes centros são habilitados pelo Ministério da Saúde como Serviços de Referência em Doenças Raras ou Serviços de Atenção Especializada. O atendimento é pelo SUS, com encaminhamento da rede de atenção básica.

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Publicações mais relevantes

Timeline de publicações
0 papers (10 anos)
#1

PDE4B deficiency aids macrophage differentiation and contributes to Cryptococcus neoformans brain infection.

PLoS pathogens2026 Mar

Cryptococcal meningitis is a fatal complication. Macrophages have been proposed to function as candidate "Trojan horse" cells, transferring Cryptococcus neoformans (C. neoformans) into the brain. The mechanisms of Trojan horses in cryptococcal meningitis are largely elusive. In this study, we performed scRNA-Seq on immune cells infiltrating the brain in a murine model of cryptococcal meningitis. Bioinformatics analysis revealed that phosphodiesterase 4B (PDE4B) is a candidate regulator associated with C. neoformans infected-macrophage. C. neoformans increases the total level of PDE4B in macrophages. However, virulent strains with increased production of melanin paradoxically decreased PDE4B expression in macrophages, implying that PDE4B in macrophages may be negatively associated with C. neoformans invasion. PDE4B inhibition increased Arg1, CXCR4 and CCR7 expression in macrophages, a process regulated by the cAMP/PKA signaling pathway. As expected, PDE4B inhibitors promote the ability of C. neoformans infected-macrophages to cross the blood-brain barrier (BBB) in vitro. Similarly, PDE4B inhibitors or PDE4B knockout increase the fungal burden in the brain, which is, at least partially, rescued by macrophage depletion, and adoptive transfer experiments further support macrophage-mediated fungal delivery to the brain. In contrast, PDE4B activation reduces fungal burden in the brain, including when administered after infection onset. Overall, this study revealed that PDE4B functions as an important regulator of macrophage functional programming during infection and supports a macrophage-mediated dissemination mechanism contributing to brain invasion, and is a potential therapeutic target for cryptococcal meningitis.

#2

Chronic Histamine Exposure Promotes Melanogenesis via ORAI1-STIM1-Mediated Calcium Signaling Remodeling.

International journal of molecular sciences2026 Feb 22

Post-inflammatory hyperpigmentation (PIH) is a common pigmentary disorder characterized by excessive melanin production following skin inflammation. Histamine, a key inflammatory mediator, is known to stimulate melanogenesis via H2 receptors; however, the underlying calcium (Ca2+) signaling mechanisms remain largely unexplored. In this study, we investigated the role of the ORAI1-STIM1 complex in histamine-induced melanogenesis using B16F10 melanoma cells and normal human epidermal melanocytes (NHEMs). Histamine (10-30 μM) significantly increased melanin content (2.5-2.8-fold), an effect specifically abolished by the H2 antagonist famotidine. Notably, while acute histamine application failed to trigger immediate Ca2+ influx, chronic exposure significantly enhanced store-operated Ca2+ entry (SOCE) capacity by approximately 2.8-fold, providing evidence for a functional remodeling of the Ca2+ signaling machinery. Histamine-induced melanogenesis was significantly suppressed by intracellular Ca2+ chelation, pharmacological inhibition of ORAI1 (BTP-2 or Synta-66), and siRNA-mediated silencing of ORAI1 or STIM1, but not ORAI2, ORAI3, or STIM2. Our findings demonstrate that chronic histamine exposure drives hyperpigmentation through ORAI1-STIM1-mediated SOCE remodeling, establishing this complex as a promising therapeutic target for the treatment of PIH and related inflammatory pigmentary disorders.

#3

Insights from Computational Dynamic Active Site Mapping into Substrate Recognition and Mutation-Induced Dysfunction in Human Tyrosinase.

International journal of molecular sciences2026 Feb 18

The ability of enzymes to recognize and process structurally diverse substrates is fundamental to metabolic flexibility and biological regulation. In melanin biosynthesis, human tyrosinase (Tyr) catalyzes the oxidation of several chemically distinct intermediates, including L-tyrosine, L-DOPA, DHICA, and DHI. Although its catalytic chemistry is well established, the structural basis of substrate selectivity and how it is altered by disease-associated mutations remains unclear. Using molecular docking and molecular dynamics simulations, we mapped the Tyr active site and identified 23 evolutionarily conserved residues that mediate multi-substrate recognition and binding. Across all substrates, binding induces coordinated conformational responses, particularly within an anchoring region (334-347) that provides electrostatic and hydrophobic steering, and a flexible gating loop (374-386) that modulates access and stabilizes bound intermediates. The OCA1B-associated P406L mutation, although distant from the catalytic core, disrupts long-range dynamic coupling and impairs loop flexibility, while 25 ClinVar-listed genetic variants at substrate-interacting residues weaken active-site organization, underscoring the sensitivity of Tyr's dynamic network to perturbation. Integrating these findings, we propose an ordered multi-substrate binding mechanism in which substrates are first guided by the anchoring region, then aligned by the universal triad, and finally refined through loop-mediated, substrate-specific contacts. Our work suggests a dynamic framework that could be useful for understanding human tyrosinase catalysis, genetic mutation impact, and future engineering strategies.

#4

Dihydroxyhexanoic acid biosynthesis controls turgor in pathogenic fungi.

Science (New York, N.Y.)2026 Feb 12

Many plant pathogenic fungi penetrate host surfaces mechanically, using turgor pressure generated by specialized infection cells called appressoria. These appressoria develop semipermeable cell walls and accumulate osmolytes internally to create turgor by osmosis. Although melanin is known to be important for turgor generation, the mechanism underlying wall semipermeability remains unclear. By using reverse genetics, we identified that the enzymes PKS2 and PBG13 are required for forming the semipermeable barrier in fungi causing anthracnose and rice blast diseases. These enzymes synthesize 3,5-dihydroxyhexanoic acid polymers that are essential for pathogenicity. These polymers reduce cell wall permeability and generate turgor, independently of melanization. Our findings uncover a mechanism of fungal turgor generation, linking enzyme function to pathogen penetration and disease potential, presenting new targets for disease control.

#5

Preparation and Evaluation of the Synergistic Benefits of a Glycoside-Pyrone-Based Multifunctional System as a Possible Regulator for Melanogenesis.

Macromolecular bioscience2026 Feb

The skin is constantly exposed to external factors throughout a person's life, ultraviolet (UV) radiation being one of the most harmful. The primary defence against UV-induced damage is skin pigmentation, which is achieved through the synthesis of melanin. However, overproduction of melanin can lead to skin disorders such as pigment spots, melasma, and even melanoma. Therefore, the present study aimed to obtain a new multifunctional bioactive system (MBS) starting from a supramolecular co-assembled gel (SG) based on amino acids and short peptides enhanced with a glycoside-pyrone-based complex (arbutin-kojic acid), presenting an inhibitory effect on peroxidase and implicitly controlling melanin production. The MBS gel was physicochemically analyzed using FTIR to observe changes in its chemical structure after exposure to 4°C and 25°C. The results indicate that MBS remains stable for up to 12 weeks without chemical changes in structure when stored at 4°C. The potential applicability was evaluated by antioxidant activity, where the gel exhibited above 85% scavenging activity of DPPH· free radicals. The MBS displays synergistically strong ability to inhibit the catalytic activity, functioning as an uncompetitive inhibitor that binds specifically to the enzyme-substrate complex. The in vivo biosafety of the MBS was determined at 24 h and 7 days after rat administration. The hematological and biochemical parameters show that the MBS system is safe and biocompatible both after 24 h and after 7 days. The overall findings suggest that the MBS gel has promising potential as a regulator of melanogenesis by inhibiting skin melanin synthesis.

Publicações recentes

Ver todas no PubMed

📚 EuropePMCmostrando 199

2026

Seasonal climatic variability shapes immune responses and infection risks in the common bluetail damselfly.

Oecologia
2026

Combined analysis of the triglyceride-glucose index and melanin-concentrating hormone in metabolic dysfunction-associated fatty liver disease: a machine learning-based study.

Frontiers in nutrition
2026

Mechanism of Inonotus hispidus in treating melasma: integrated in vivo, in vitro, network pharmacology and untargeted metabolomics investigation.

Journal of ethnopharmacology
2026

PDE4B deficiency aids macrophage differentiation and contributes to Cryptococcus neoformans brain infection.

PLoS pathogens
2026

A furanochromone derivative, visnagin stimulates melanogenesis via the activation of cAMP/PKA/CREB pathway.

European journal of pharmacology
2026

Chronic Histamine Exposure Promotes Melanogenesis via ORAI1-STIM1-Mediated Calcium Signaling Remodeling.

International journal of molecular sciences
2026

Insights from Computational Dynamic Active Site Mapping into Substrate Recognition and Mutation-Induced Dysfunction in Human Tyrosinase.

International journal of molecular sciences
2026

Clematis Tangutica (Maxim.) Korsh. extracts promote melanogenesis via PKA/CREB activation and multi-cytokine inhibition: A novel dual targeting strategy for vitiligo therapy.

Phytomedicine : international journal of phytotherapy and phytopharmacology
2026

Dihydroxyhexanoic acid biosynthesis controls turgor in pathogenic fungi.

Science (New York, N.Y.)
2026

Atopic Dermatitis Accelerates Skin Physiological Functional Decline and Visible Aging, Suppressed by Skincare Habits.

Journal of cosmetic dermatology
2026

Preparation and Evaluation of the Synergistic Benefits of a Glycoside-Pyrone-Based Multifunctional System as a Possible Regulator for Melanogenesis.

Macromolecular bioscience
2026

Neuromelanin-sensitive MRI as a biomarker for individualized deep brain stimulation in Parkinson's disease: Associations between substantia nigra imaging and therapeutic outcomes.

Parkinsonism & related disorders
2025

Tricyclazole alleviates Fonsecaea pedrosoi-induced immune suppression of neutrophils by inhibiting DHN-melanin biosynthesis.

Frontiers in cellular and infection microbiology
2026

Potential Bioactive Function of Microbial Metabolites as Inhibitors of Tyrosinase: A Systematic Review.

International journal of molecular sciences
2026

Monopolar Radiofrequency for Facial Hyperpigmentation Treatment: An Integrated Retrospective Clinical Trial and Ex Vivo Study.

International journal of molecular sciences
2026

Biological Roles of Melanin and Natural Product-Derived Approaches for Its Modulation.

International journal of molecular sciences
2026

Human Alpha-1 Antitrypsin Suppresses Melanoma Growth by Promoting Tumor Differentiation and CD8+ T-Cell-Mediated Immunity.

Biomolecules
2025

Melanin and Neuromelanin in Humans: Insights Across Health, Aging, Diseases, and Unexpected Aspects of Fungal Melanogenesis.

Biomolecules
2026

Testing dopaminergic markers of problematic social media use using neuromelanin-sensitive MRI.

Psychiatry research. Neuroimaging
2026

RNAi-based screen for pigmentation in Drosophila melanogaster reveals regulators of brain dopamine and sleep.

iScience
2026

Levodopa Suppresses Choroidal Neovascularization Through a Tyrosinase-Dependent Dual Mechanism.

Investigative ophthalmology & visual science
2026

Evaluation of Melanin Changes in Acute Vogt-Koyanagi-Harada Disease Using Polarization-Sensitive Optical Coherence Tomography.

Investigative ophthalmology & visual science
2026

Drug-Excipient Interaction-Mediated Supersaturated Emulsion Gel for Enhanced Intradermal Delivery of Tranexamic Acid.

Molecular pharmaceutics
2026

Peroxisome Membrane Protein PEX16 Inhibits Melanogenesis by Inhibiting the Wnt/β-Catenin Signalling Pathway.

Experimental dermatology
2026

Vesicular Transport Mediated by Endoplasmic Reticulum Stress Sensor BBF2H7 Orchestrates Melanin Production During Melanogenesis.

International journal of molecular sciences
2025

Protective Effect of Peony (Paeonia ostii) Flower Extract Against Tape Stripping-Induced Skin Barrier Impairment in Mice.

Molecules (Basel, Switzerland)
2026

Photodistributed Hyperpigmentation Associated With COVID-19 Vaccination.

Journal of drugs in dermatology : JDD
2026

[Hormones and skin pigmentation: fundamentals and clinical relevance].

Dermatologie (Heidelberg, Germany)
2026

Genome-wide association study reveals genetic architecture and evolution of human retinal pigmentation.

Science advances
2026

Comparative transcriptomics identifies key genes and pathways underlying the early skin coloration in leopard coral grouper (Plectropomus leopardus).

Comparative biochemistry and physiology. Part D, Genomics & proteomics
2025

Gut microbiome associated with melanin deposition by supporting energy metabolism in Sichuan mountainous black-bone chickens.

Frontiers in microbiology
2025

The Diatom Odontella aurita Modulates Melanogenesis in B16-F0 Cell Line.

Antioxidants (Basel, Switzerland)
2025

Synergistic interplay between UV and urban particulate matter exposure induces melanocyte senescence and contributes to human skin aging.

Scientific reports
2025

Revisiting the alpha-synuclein paradox in melanoma-Parkinson's disease connection: more than a tale of two cell fates.

Cellular and molecular life sciences : CMLS
2026

Activation of lateral hypothalamic melanin-concentrating hormone neurons mediates sex-specific patterns of methamphetamine self-administration in rats.

Behavioural brain research
2025

Generation and ophthalmological characterization of oculocutaneous albinism type 1 pig models by selection-free genome editing.

Scientific reports
2025

6-Isoprenylindole-3-carboxylic Acid with an Anti-Melanogenic Activity from a Marine-Derived Streptomyces sp. APA-053.

Marine drugs
2026

Decoding the Mechanisms of Pigment Reduction and Skin Rejuvenation Induced by Picosecond Laser: Insights From a Porcine Model.

Lasers in surgery and medicine
2026

Near-Infrared Autofluorescence in Non-Infectious Uveitis: A Review.

Ocular immunology and inflammation
2026

CRISPR/Cas-mediated polyphenol oxidase gene knockout in potato reveals divergent roles in resistance to bacterial wilt and late blight.

Plant science : an international journal of experimental plant biology
2025

The Combination of Pterocarpus marsupium Bark Extract, Pinus strobus Bark Extract, and Ascorbyl Tetraisopalmitate Inhibits Melanogenesis via Nicotinamide Nucleotide Transhydrogenase Activation.

Journal of cosmetic dermatology
2026

Keratin intermediate filaments mechanically position melanin pigments for genome photoprotection.

Nature cell biology
2026

Apoferritin-conjugated melanin nanoparticles rescue photoreceptor degeneration via dual iron chelation and ROS scavenging in dry AMD therapy.

International journal of biological macromolecules
2025

Apple oil as a source of ursolic acid for the treatment of hyperpigmentary disorders with molecular and clinical evaluation.

Scientific reports
2025

Consumption of Unprocessed and Ultraprocessed Foods in Adolescents with Obesity: Associations with Neuroendocrine Mediators of Appetite Regulation and Binge Eating Symptoms.

Nutrients
2025

Mechanistic Insights into Anti-Melanogenic Effects of Fisetin: PKCα-Induced β-Catenin Degradation, ERK/MITF Inhibition, and Direct Tyrosinase Suppression.

International journal of molecular sciences
2025

Microneedle Patch Delivering Multifunctional Melanin-like Nanoparticles for Vitiligo Remission and Repigmentation.

ACS applied materials & interfaces
2025

Early skin seeding regulatory T cells modulate PPARγ-dependent skin pigmentation.

Nature communications
2026

Effects of wavelength, fluence, irradiance, and irradiation mode of visible light on melanogenesis in B16F10 melanoma cells.

Journal of photochemistry and photobiology. B, Biology
2026

UVB enhances SLC6A15-mediated phenylalanine transport to promote melanogenesis.

Journal of photochemistry and photobiology. B, Biology
2025

Postmortem Retinal Structural and Metabolic Analysis After Human Embryonic Stem Cell-derived Retinal Pigment Epithelium Transplantation in a Patient With Stargardt Disease.

Journal of vitreoretinal diseases
2025

Beyond Parkinson's Disease: A Narrative Review of Neuromelanin MRI in Neurodegenerative Diseases.

Journal of neuroimaging : official journal of the American Society of Neuroimaging
2026

Modulating cerebrospinal fluid dynamics using pulsed photobiomodulation.

Brain stimulation
2025

Discovery of a bell-shaped dose response curve to melanin-concentrating hormone in the 3T3-L1 adipocyte model: low-dose MCH facilitates adipogenesis.

Adipocyte
2025

Hormonal Crosstalk in Melasma: Unraveling the Dual Roles of Estrogen and Progesterone in Melanogenesis.

International journal of molecular sciences
2025

The very-long-chain (3R)-3-hydroxyacyl-CoA dehydratase Phs1 regulates ATP levels and virulence in Cryptococcus neoformans.

BMC microbiology
2025

Hippo signaling regulates cuticle pigmentation and dopamine metabolism in Drosophila.

bioRxiv : the preprint server for biology
2025

Atomic structure and in situ visualization of native PMEL lamellae in melanosomes.

Nature communications
2026

Aloe Kanti, a natural anti-aging gel, modulates exogenous insult- and aging-induced aberrations in keratinocytes, dermal fibroblasts, melanocytes, and protects Caenorhabditis elegans from UVB photoaging.

Fitoterapia
2026

Heat Stress Modulates WDR5-Mediated H3K4me3 Modification to Induce Melanogenesis via Activating CX3CL1/CX3CR1 Axis.

Advanced science (Weinheim, Baden-Wurttemberg, Germany)
2025

Drug repurposing and AI-driven discovery of tyrosinase inhibitors, emerging strategies for skin disorders: A review.

International journal of biological macromolecules
2026

6-Bromo-Indirubin-3'-Oxime (6BIO) mitigates oxidative stress and immune dysregulation to promote melanocyte regeneration in vitiligo.

Free radical biology & medicine
2025

High-quality genome assemblies provide new insights into the genomic architecture, adaptation, and virulence of neurotropic dematiaceous fungi implicated in cerebral phaeohyphomycosis.

Medical mycology
2025

Microbiome and metabolomics analyses of the effect of heat-sensitive moxibustion on allergic rhinitis in rats.

Frontiers in immunology
2026

The Relationship Between Neuromelanin, Glutamate, and GABA in First-Episode Psychosis: A Multimodal Magnetic Resonance Imaging Study.

Biological psychiatry. Cognitive neuroscience and neuroimaging
2025

A Potential Role of Natural Bioactive Compounds Found in Food in the Prevention of Idiopathic Parkinson's Disease.

Nutrients
2025

Multi-Omics Analysis of the Potential Mechanisms of Skin Albinism in Edangered Percocypris pingi: Abnormal Ubiquitination and Calcium Signal Inhibition.

Cells
2025

Over-Represented Senescent Keratinocytes in Hyperpigmented Spots Promote Melanocyte Activation via IGFBP3 and NGF.

International journal of molecular sciences
2025

inhibitory effect of hyperin on Staphylococcus aureus pathogenicity though interactions with sortase A and sortase B.

Scientific reports
2026

Early brain-wide disruption of sleep microarchitecture in amyotrophic lateral sclerosis.

The Journal of clinical investigation
2026

Reversing fibroblast-to-myofibroblast transition using surface-engineered nanoparticles to potentially ameliorate fibrotic diseases.

Biomaterials
2025

The DOPA scaffold: Tracing catechol chemistry from prebiotic earth to cognitive agency.

Progress in biophysics and molecular biology
2026

Assessment of Galleria mellonella as an in vivo virulence model for Brachyspira species associated with avian intestinal spirochaetosis.

Microbial pathogenesis
2025

Potassium phosphite effectively controls rubber tree anthracnose by inhibiting melanin biosynthesis of Colletotrichum siamense.

Pesticide biochemistry and physiology
2025

KT-939: A Next-Generation Human Tyrosinase Inhibitor With Superior Efficacy for the Safe Management of Hyperpigmentation.

Journal of cosmetic dermatology
2025

Targeting Melanogenesis with Postbiotics: An Integrated Zebrafish-Based Assessment of Lactobacillus salivarius BGHO-1 and Lactobacillus paracasei BGSJ2-8.

Molecules (Basel, Switzerland)
2026

Insight Into the Cellular Activities of Tranexamic Acid as an Option for Melasma Treatment.

Cell biology international
2025

Concentration-dependent effects of bacterial melanin on new superoxide-producing associates in rat tissues: a rotenone neurotoxic model of parkinson's disease.

BMC pharmacology & toxicology
2025

The Impact of Sleep Quality on Skin Color.

Indian dermatology online journal
2025

The efficacy of topical treatments for acanthosis nigricans: a systematic review of randomized controlled trials.

Frontiers in medicine
2025

Melanogenesis inhibition and anti-inflammation is essential for pigment-clearance in melasma treated by low-fluence Q-switched nd: YAG 1064 nm laser.

Lasers in medical science
2025

In vitro bioactivities and formulation stability of Houttuynia cordata essential oil for cosmetic applications.

Scientific reports
2025

Influence of Nitrative Stress on the Synthesis of Neuromelanin Model Systems.

ACS chemical neuroscience
2026

Genetic Pigmentary Disorders: From Molecular Mechanisms to Clinical Manifestations.

The Journal of dermatology
2025

The extract of dendrobium Coelonin inhibits PIH induced by AFR CO2 fractional laser combined with UV-B.

Photochemical & photobiological sciences : Official journal of the European Photochemistry Association and the European Society for Photobiology
2026

The correlation of pigment content between dermoscopy and histopathology in basal cell carcinoma: A retrospective study.

Indian journal of dermatology, venereology and leprology
2026

Antifungal mechanisms of amaryllidaceous alkaloids lycorine and narciclasine against the rice blast fungus Magnaporthe oryzae.

Pest management science
2025

Induction of Melanin Synthesis by Pueraria lobata Leaves in B16 Murine Melanoma Cells and Three-Dimensional Human Skin Equivalent.

Biological & pharmaceutical bulletin
2025

Optimizing cinnamophilin delivery via SNEDDS for enhanced anti-melanogenic activity: A comprehensive evaluation of skin safety, permeability, and tyrosinase inhibition.

International journal of pharmaceutics
2025

Pharmacological Potential and Molecular Targets of Tetrahydrofurofuranoid Lignans From Magnoliae Flos.

Drug design, development and therapy
2025

BLSAM-TIP: Improved and robust identification of tyrosinase inhibitory peptides by integrating bidirectional LSTM with self-attention mechanism.

PloS one
2025

Alterations of Photoreceptor Synaptic Ribbons in the Retina of a Human Patient With Oculocutaneous Albinism Type 1 (OCA1).

Investigative ophthalmology & visual science
2025

The Incorporation of Melanosomes by Senescent Keratinocytes Causes the Accumulation of Melanin due to Decreased Energy Metabolism.

Pigment cell & melanoma research
2025

Recent advances in gene delivery for melanocyte-associated disorders.

Advanced drug delivery reviews
2025

Regulation of melanogenesis via ubiquitin-proteasome system and autophagy by 3,3,5-trimethylcyclohexyl succinate dimethylamide and tranexamic acid.

Journal of dermatological science
2025

Novel Dual-Action Whitening Peptides Derived from Tea Protein Hydrolysates.

Journal of agricultural and food chemistry
2025

Functional and Morphological Plasticity of the Endolysosomal System: Pigment Organelles at the Crossroads of Physiology and Pathology.

Biology of the cell
2025

L-cysteine inhibits the in vitro and in vivo growth of Alternaria alternata via disrupting cell membrane integrity and compromising cell wall structure.

Microbial pathogenesis
2025

Noninvasive Assessment of Melasma Pathological Features: Side-By-Side Comparison of Two-Photon Microscopy and Reflectance Confocal Microscopy.

Pigment cell & melanoma research
2025

Galleria mellonella possesses the essential nutritional needs to host the fastidious Huanglongbing bacterial pathogen 'Candidatus Liberibacter asiaticus'.

Communications biology
2025

Catecholaminergic nucleus integrity and Alzheimer's pathology, symptoms, and progression.

Alzheimer's & dementia : the journal of the Alzheimer's Association
2025

In Vitro Characterization of Centella asiatica Extracellular Vesicles and Their Skin Repair Effects in a UVB-Irradiated Mouse Model.

International journal of molecular sciences
2025

Effect of Tigecycline on the Homeostasis of Human Epidermal Melanocytes and Fibroblasts.

International journal of molecular sciences
2025

Amyloid Peptide Nanofibrils Promote Efficient Neurotransmitter Oxidation and Serve as Scaffolds for Melanin Production.

Angewandte Chemie (International ed. in English)
2025

Neuromelanin-Sensitive MRI Contrast and Chronic Depression in Young Women.

JAMA network open
2026

Single-cell RNA sequencing reveals the transcriptomic landscape of and potential targets for large and giant congenital melanocytic naevi.

The British journal of dermatology
2025

Modeling Midbrain and Brainstem Neuromelanins to Characterize Metal Binding and Associated MRI Contrast in Parkinson's and Alzheimer's Diseases.

Angewandte Chemie (International ed. in English)
2025

Association Between Pigmentation Heritage and Susceptibility to Experimentally Induced Myopia: Crossbreeding Insights From Albino and Pigmented Guinea Pigs.

Investigative ophthalmology & visual science
2026

Investigating the effects and mechanisms of Vernonia anthelmintica (L.) willd., seed extracts on melanogenesis and vitiligo treatment based on multi-omics and network pharmacology.

Journal of ethnopharmacology
2025

A Spatially Resolved View on the Aging Substantia nigra: An Exploratory Proteomic Study.

Advanced biology
2025

Ultraviolet-tyrosinase cascade caged antisense oligonucleotide for precise treatment of hyperpigmentation.

Journal of controlled release : official journal of the Controlled Release Society
2025

Melanosome Transport and Processing in Skin Pigmentation: Mechanisms and Targets for Pigmentation Modulation.

International journal of molecular sciences
2025

Dual Modulatory Effects of Phytochemicals from Iris ×germanica L. var. florentina Dykes Rhizome Extract on Melanogenesis.

Molecules (Basel, Switzerland)
2025

Histopathological Characteristics and Multi-Omics Analysis of Ocular Pigmentation Defects in Albino Percocypris pingi.

Cells
2026

Hemispheric Asymmetry of Neuromelanin-Iron Dysfunction in the Substantia Nigra: MRI-Based Evidence for Lateralized Motor Onset in Early-Stage Parkinson's Disease.

Journal of magnetic resonance imaging : JMRI
2026

A Core-Shell Structured Microneedle Patch With Adjustable Release of Kinetically for the Treatment of Melasma.

Advanced healthcare materials
2025

Efficacy of Intense Pulsed Light AOPT-LTL Technique in the Treatment of Melasma: An In Vivo and Clinical Study.

Journal of cosmetic dermatology
2026

A multifunctional L-arginine-linked melanin Nanozyme for Theranostic applications in liver fibrosis: Non-invasive dual-modal imaging and reactive oxygen species scavenging for Pyroptosis suppression.

Journal of colloid and interface science
2025

Targeting Melanin Heterogeneity in Metastatic Melanoma: A Dual-Tumour Mouse Melanoma Model.

Experimental dermatology
2026

A model for de novo pigmentation of amelanotic retinal pigment epithelial cells.

Acta ophthalmologica
2025

Reduced Brain Iron and Striatal Hyperdopaminergia in Schizophrenia: A Quantitative Susceptibility Mapping MRI and PET Study.

The American journal of psychiatry
2025

Sleep in neurodegenerative diseases: A focus on melatonin, melanin-concentrating hormone and orexin.

Journal of neuroendocrinology
2025

CSF markers of neuroinflammation, synaptic dysfunction and [18F]DOPA-PET in Parkinson's disease.

Parkinsonism & related disorders
2025

Repurposing the Antibiotic D-Cycloserine for the Treatment of Hyperpigmentation: Therapeutic Potential and Mechanistic Insights.

International journal of molecular sciences
2025

Unculturable bacteria exploit a secretory protein to antagonize insect melanization for persistent infection.

mBio
2025

Ubiquitinome profiling identifies USP13-CMAS axis as critical regulator of hair follicular melanogenesis via K48-linked polyubiquitination.

Cellular signalling
2025

Natural dual inhibitor isorhamnetin-3-O-neohespeidoside targets tyrosinase and MC1R for skin pigmentation management.

Scientific reports
2025

Neuromelanin Contrast Optimization and Improved Visualization of the Substantia Nigra in a 3D Gradient-Echo Sequence With Magnetization Transfer.

NMR in biomedicine
2025

Cinnamic Acid Derivatives as Potential Melanogenesis Inhibitors for Use in Cosmetic Products.

Chemistry & biodiversity
2026

Integrated evaluation of Nigrosome 1 sign, neuromelanin-sensitive MR and iron deposition.

Japanese journal of radiology
2025

Aspergillus fumigatus dsRNA virus promotes fungal fitness and pathogenicity in the mammalian host.

Nature microbiology
2025

Intrinsic and extrinsic factors affecting the evolution of virulence in the HIV-associated opportunistic human fungal pathogen Cryptococcus neoformans.

Virulence
2025

New Insights into the Synergistic Bioactivities of Zingiber officinale (Rosc.) and Humulus lupulus (L.) Essential Oils: Targeting Tyrosinase Inhibition and Antioxidant Mechanisms.

Molecules (Basel, Switzerland)
2025

Melanin-Concentrating Hormone (MCH): Role in Mediating Reward-Motivated and Emotional Behavior and the Behavioral Disturbances Produced by Repeated Exposure to Reward Substances.

International journal of molecular sciences
2025

LRRK2-mutant microglia and neuromelanin synergize to drive dopaminergic neurodegeneration in an iPSC-based Parkinson's disease model.

Communications biology
2025

Endogenous Pigment Mimicking Engineered Nanovesicle Targets Extrasynaptic NMDA Receptors against Ca2+-Mediated Excitotoxicity in Alzheimer's Disease.

ACS applied materials & interfaces
2025

Therapeutic effects of topical Mycophenolate mofetil on hydroquinone-induced depigmentation in Guinea pigs and mice.

Annals of medicine
2025

Potential of ononin as a safe and effective anti-melanogenic agent: In vitro and zebrafish embryo study.

European journal of pharmacology
2025

Disrupting the MC1R/α-MSH-pCREB-MITF Axis: Rhein-based PROTAC D16 as a Potent Melanogenesis Inhibitor.

Chemistry & biodiversity
2025

Beyond the Skin Surface: Melanocyte Biology and the Spectrum of Health Inequities.

The Journal of investigative dermatology
2025

Neuromelanin-induced cellular stress and neurotoxicity in the pathogenesis of Parkinson's disease.

Apoptosis : an international journal on programmed cell death
2025

Riboflavin inhibits growth and reduces virulence of Cryptococcus neoformans in vitro by membrane disruption and excessive accumulation of reactive oxygen species and exhibits efficacy against pulmonary cryptococcosis and meningitis.

Virulence
2025

Full length transcriptomic profiling reveals insights into the white coat phenotype in Waardenburg syndrome mice harboring the Mitf R324del mutation.

Scientific reports
2025

Exploring the regulatory role of long non-coding RNAs in pigmentation in juvenile Plectropomus leopardus.

Scientific reports
2025

Lipidome Complexity in Physiological and Pathological Skin Pigmentation.

International journal of molecular sciences
2025

The Integrated Function of the Lateral Hypothalamus in Energy Homeostasis.

Cells
2026

Corticotropin-Releasing Hormone (CRH) in Murine Narcolepsy: What Do Genetic and Immune Models Tell Us?

Journal of sleep research
2025

Treatment of hypopigmented scar with autologous skin cell suspension delivered through fractional ablative laser-assisted drug delivery does not lead to short-term re-pigmentation.

Burns : journal of the International Society for Burn Injuries
2026

Anatomy of a bioengineered human pigmented skin equivalent to provide fundamental insights into skin tone melanin dynamics.

Journal of anatomy
2025

Daidzin suppresses melanogenesis through ERK and AKT signaling pathways mediated MITF proteasomal degradation.

Experimental and molecular pathology
2025

Associational study of neonatal hearing screening results and common metabolic disorders.

International journal of pediatric otorhinolaryngology
2025

Exogenous ochronosis by hydroquinone is not caused by inhibition of homogentisate dioxygenase but potentially by tyrosinase-catalysed metabolism of hydroquinone.

The British journal of dermatology
2025

Mitochondrial homeostasis: Exploring their impact on skin disease pathogenesis.

Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie
2025

Endogenous tyrosinase-catalyzed therapeutics.

Nature communications
2025

Effect of dual optical pulse scheme with supra and subthreshold fluences on laser micro ablation of pigment.

Scientific reports
2025

Exploring the molecular mechanism of Polygonum multiflorum in treating androgenic alopecia based on the methods of bioinformatics and molecular docking.

Medicine
2025

Methylcobalamin-loaded ultra-flexible liposomes: a nanocarrier approach for modulating melanocyte homeostasis and addressing pigmentation imbalances.

International journal of pharmaceutics
2025

Genetics of Skin, Hair, and Eye Color in Human Pigmentation Disorders.

Annals of human genetics
2025

Unsupervised SAM segmentation of zebrafish body: Application to melanin analysis.

Environmental pollution (Barking, Essex : 1987)
2025

Cryo-EM of wild-type and mutant PMEL amyloid cores reveals structural mechanism of pigment dispersion syndrome.

Nature communications
2025

Oxyresveratrol suppressed melanogenesis, dendrite formation, and melanosome transport in melanocytes via regulation of the MC1R/cAMP/MITF pathway.

Scientific reports
2025

Effect and Safety of Skincare Regimens Containing a Multi-Molecular Hyaluronic Acid Complex for Recovery After Ablative Fractional CO2 Laser: A Prospective, Randomized, Controlled Trial.

Journal of cosmetic dermatology
2025

Prospective Isolation According to Melanin Pigment Content of Melanoma Cells With Heterogeneous Potentials for Disease Propagation.

Pigment cell & melanoma research
2025

Regional neuromelanin reduction in the substantia nigra in different subtype of Parkinson's disease.

Parkinsonism & related disorders
2025

Blue light regulates jasmonic acid synthesis via CRY1a and boosts antioxidant enzymes activity in Solanum lycopersicum to resist Botrytis cinerea.

Plant cell reports
2025

Andrographolide and Mahua Oil-Infused Emulgels: A Comprehensive QbD, In Vitro, and In Silico Strategy for Skin Pigmentation Treatment.

Chemistry & biodiversity
2026

Melanin nanoparticles-loaded lactobacillus fermentum exosomes for targeted and visualized treatment of ulcerative colitis.

Journal of advanced research
2025

The in cellular and in vivo melanogenesis inhibitory activity of safflospermidines from Helianthus annuus L. bee pollen in B16F10 murine melanoma cells and zebrafish embryos.

PloS one
2025

Poor Diagnostic Performance of the Melanin-Binding Tracer [18 F]MEL050 in Human Melanoma Indicates Biological Heterogeneity.

Molecular imaging and biology
2025

Strabismus and nystagmus in oculocutaneous albinism: clinical perspectives, diagnosis, and role of neurotransmitters.

Neurogenetics
2025

Anti-Melanogenesis Activity of Peptides from Shark (Mustelus griseus) Skin on B16F10 Melanocytes and In vivo Zebrafish Models.

Applied biochemistry and biotechnology
2025

Clinical Investigation of Tyrosinase Inhibitors: Past, Present, and Future.

Drug development research
2025

First-in-human PET imaging and evaluation of melanin-targeted [18F]DMPY2 in malignant melanoma patients.

Theranostics
2025

Effects of Resveratrol Derivatives on Melanogenesis and Antioxidant Activity in B16F10 Cells.

International journal of molecular sciences
2025

Thiamidol: A Breakthrough Innovation in the Treatment of Hyperpigmentation.

Journal of drugs in dermatology : JDD
2025

Biosynthesis of Melanin with Engineered Probiotics for Oral Treatment of Ulcerative Colitis.

ACS nano
2025

Hair follicle-derived melanocyte transplant as a promising treatment strategy for vitiligo.

Stem cell research & therapy
2025

Plant-Derived Monomers for Grey Hair Reversal Through Upregulation of Melanogenesis and Tyrosinase Activity.

Journal of cellular and molecular medicine
2025

Integrated RNA interference and RNA-sequencing analysis of the effects of laccase2 on cuticular pigmentation and survival in Halyomorpha halys (Stål) (Hemiptera: Pentatomidae).

Pest management science
2025

Safety and efficacy of platelet-rich plasma in the treatment of periorbital skin photoaging.

Journal of cosmetic and laser therapy : official publication of the European Society for Laser Dermatology
2025

TDP-43 overexpression in the hypothalamus drives neuropathology, dysregulates metabolism and impairs behavior in mice.

Acta neuropathologica communications
2025

Alteration of Hair Melanin in Patients With Mowat-Wilson Syndrome: The Role of the ZEB2 Gene in Regulating Melanogenesis Through SLC45A2.

Pigment cell & melanoma research
2025

Rubia cordifolia L. extract ameliorates vitiligo by inhibiting the CXCL10/CXCL9/STAT1 signaling pathway.

Journal of ethnopharmacology
2025

Hyperbranched polymer dots enhance hair follicle regeneration via Wnt/β-catenin activation: A drug-free nanozyme-based approach to hair growth therapy.

Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie
2025

Influence of melanin and macrophage activation on hearing loss in SLC26A4 deficient mice.

Neurobiology of disease
2025

Linking polygenic risk scores to dopaminergic neuron loss using neuromelanin-sensitive imaging.

Brain : a journal of neurology
2025

Potential Cutaneous Applications of Boesenbergia rotunda Extract Based on Its In Vitro Anti-Melanogenic and Anti-Fibroproliferative Properties.

International journal of molecular sciences
2025

Rtf1 HMD domain facilitates global histone H2B monoubiquitination and regulates morphogenesis and virulence in the meningitis-causing pathogen Cryptococcus neoformans.

eLife
2025

Inhibition effects of Eucalyptus globules Labill. essential oil against tyrosinase.

Scientific reports
2025

Antimicrobial Agent Trimethoprim Influences Chemical Interactions in Cystic Fibrosis Pathogens via the ham Gene Cluster.

ACS chemical biology
2025

Unraveling the Role of Functional Amyloids and Amyloid Peptides in Disease Detection.

Protein and peptide letters
2025

Neuromelanin and selective neuronal vulnerability to Parkinson's disease.

Trends in neurosciences
2025

Characterisation of localised pigment accumulation in brains of eastern grey kangaroos (Macropus giganteus) after clinical disease due to chronic Phalaris species toxicosis.

Australian veterinary journal
2025

Bioactive oligopeptides and the application in skin regeneration and rejuvenation.

Journal of applied biomaterials & functional materials
2025

A Narrowband 635 nm Autofluorescence Peak in Albino Mouse Eyes Found With Multi-Modal Imaging Reveals the Presence of Protoporphyrin IX in the Choroid.

Investigative ophthalmology & visual science
2025

Melanin concentrating hormone-sleep pressure loop regulates melanin degradation through both autophagic degradation and lysosomal hydrolysis in zebrafish.

The Journal of biological chemistry
2025

Correlation between persistent changes in ciliary dynamics in the FrA and depressive-like behavior.

Biochemical and biophysical research communications
2025

Convergence of Cannabis and Psychosis on the Dopamine System.

JAMA psychiatry
2025

Uncovering the bioactive constituents and their mechanisms of the Forsythiae Fructus against hyperpigmentation using a combined strategy integrating cell-specific extraction, plasma pharmaceutical chemistry and network pharmacology.

Journal of pharmaceutical and biomedical analysis

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. PDE4B deficiency aids macrophage differentiation and contributes to Cryptococcus neoformans brain infection.
    PLoS pathogens· 2026· PMID 41805786mais citado
  2. Chronic Histamine Exposure Promotes Melanogenesis via ORAI1-STIM1-Mediated Calcium Signaling Remodeling.
    International journal of molecular sciences· 2026· PMID 41752190mais citado
  3. Insights from Computational Dynamic Active Site Mapping into Substrate Recognition and Mutation-Induced Dysfunction in Human Tyrosinase.
    International journal of molecular sciences· 2026· PMID 41752073mais citado
  4. Dihydroxyhexanoic acid biosynthesis controls turgor in pathogenic fungi.
    Science (New York, N.Y.)· 2026· PMID 41678637mais citado
  5. Preparation and Evaluation of the Synergistic Benefits of a Glycoside-Pyrone-Based Multifunctional System as a Possible Regulator for Melanogenesis.
    Macromolecular bioscience· 2026· PMID 41655265mais citado
  6. A furanochromone derivative, visnagin stimulates melanogenesis via the activation of cAMP/PKA/CREB pathway.
    Eur J Pharmacol· 2026· PMID 41765271recente
  7. Clematis Tangutica (Maxim.) Korsh. extracts promote melanogenesis via PKA/CREB activation and multi-cytokine inhibition: A novel dual targeting strategy for vitiligo therapy.
    Phytomedicine· 2026· PMID 41720011recente
  8. Atopic Dermatitis Accelerates Skin Physiological Functional Decline and Visible Aging, Suppressed by Skincare Habits.
    J Cosmet Dermatol· 2026· PMID 41668302recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:352728(Orphanet)
  2. MONDO:0018134(MONDO)
  3. GARD:21528(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q55787760(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Alteração do metabolismo da melanina
Compêndio · Raras BR

Alteração do metabolismo da melanina

ORPHA:352728 · MONDO:0018134
Medicamentos
7 registrados
MedGen
UMLS
C5680988
Wikidata
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