Raras
Buscar doenças, sintomas, genes...
Doença de Stargardt
ORPHA:827CID-10 · H35.5CID-11 · 9B70DOENÇA RARA

A doença de Stargardt, também conhecida como Stargardt 1 (STGD1), é uma forma autossômica recessiva de distrofia retiniana que geralmente é caracterizada por uma perda progressiva da visão central associada a manchas irregulares de fundo amarelo-branco maculares e perimaculares e uma chamada lesão macular central atrófica de bronze batido.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

A doença de Stargardt, também conhecida como Stargardt 1 (STGD1), é uma forma autossômica recessiva de distrofia retiniana que geralmente é caracterizada por uma perda progressiva da visão central associada a manchas irregulares de fundo amarelo-branco maculares e perimaculares e uma chamada lesão macular central atrófica de bronze batido.

Pesquisas ativas
32 ensaios
65 total registrados no ClinicalTrials.gov
Publicações científicas
1.176 artigos
Último publicado: 2026 Apr 21
Medicamentos
2 registrados
EMIXUSTAT, METFORMIN

Tem tratamento?

2 medicamentos registrados
Ver detalhes, fases e interações →
EMIXUSTATMETFORMIN

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
13.0
Europe
Início
Adolescent
+ adult, childhood, elderly
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: H35.5
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

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

Partes do corpo afetadas

👁️
Olhos
35 sintomas
🧠
Neurológico
1 sintomas

+ 14 sintomas em outras categorias

Características mais comuns

100%prev.
Acuidade visual reduzida
90%prev.
Defeito da visão de cores
Muito frequente (99-80%)
90%prev.
Escotoma central
Muito frequente (99-80%)
90%prev.
Morfologia foveal anormal
Muito frequente (99-80%)
90%prev.
Morfologia anormal da coroide
Muito frequente (99-80%)
90%prev.
Degeneração macular
Muito frequente (99-80%)
50sintomas
Muito frequente (13)
Frequente (3)
Sem dados (34)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 50 características clínicas mais associadas, ordenadas por frequência.

Acuidade visual reduzidaReduced visual acuity
Muito frequente100%
Defeito da visão de coresColor vision defect
Muito frequente (99-80%)90%
Escotoma centralCentral scotoma
Muito frequente (99-80%)90%
Morfologia foveal anormalAbnormal foveal morphology
Muito frequente (99-80%)90%
Morfologia anormal da coroideAbnormal choroid morphology
Muito frequente (99-80%)90%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico1.176PubMed
Últimos 10 anos200publicações
Pico2025119 papers
Linha do tempo
2026Hoje · 2026🧪 2003Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

9 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Autosomal recessive.

ABCA4Retinal-specific phospholipid-transporting ATPase ABCA4Disease-causing germline mutation(s) inTolerante
FUNÇÃO

Flippase that catalyzes in an ATP-dependent manner the transport of retinal-phosphatidylethanolamine conjugates like 11-cis and all-trans isomers of N-retinylidene-phosphatidylethanolamine (N-Ret-PE) from the lumen to the cytoplasmic leaflet of photoreceptor outer segment disk membranes, where 11-cis-retinylidene-phosphatidylethanolamine is then isomerized to its all-trans isomer and reduced by RDH8 to produce all-trans-retinol. This transport activity ensures that all-trans-retinal generated fr

LOCALIZAÇÃO

MembraneEndoplasmic reticulumCytoplasmic vesicleCell projection, cilium, photoreceptor outer segment

VIAS BIOLÓGICAS (2)
The canonical retinoid cycle in rods (twilight vision)ABC-family proteins mediated transport
MECANISMO DE DOENÇA

Stargardt disease 1

An autosomal recessive form of Stargardt disease, a retinal degenerative disease characterized by macular dystrophy, progressive bilateral atrophy of the foveal retinal pigment epithelium, and accumulation of fluorescent flecks around the macula and/or in the central and near-peripheral areas of the retina. STGD1 patients typically lose central vision in their first or second decade of life.

OUTRAS DOENÇAS (8)
severe early-childhood-onset retinal dystrophyretinitis pigmentosa 19cone-rod dystrophy 3ABCA4-related retinopathy
HGNC:34UniProt:P78363
RPE65Retinoid isomerohydrolaseCandidate gene tested inTolerante
FUNÇÃO

Critical isomerohydrolase in the retinoid cycle involved in regeneration of 11-cis-retinal, the chromophore of rod and cone opsins. Catalyzes the cleavage and isomerization of all-trans-retinyl fatty acid esters to 11-cis-retinol which is further oxidized by 11-cis retinol dehydrogenase to 11-cis-retinal for use as visual chromophore (PubMed:16116091). Essential for the production of 11-cis retinal for both rod and cone photoreceptors (PubMed:17848510). Also capable of catalyzing the isomerizati

LOCALIZAÇÃO

CytoplasmCell membraneMicrosome membrane

VIAS BIOLÓGICAS (1)
The canonical retinoid cycle in rods (twilight vision)
MECANISMO DE DOENÇA

Leber congenital amaurosis 2

A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.

EXPRESSÃO TECIDUAL(Baixa expressão)
Substância negra
4.6 TPM
Hipotálamo
2.6 TPM
Brain Spinal cord cervical c-1
0.8 TPM
Próstata
0.6 TPM
Hipocampo
0.5 TPM
OUTRAS DOENÇAS (7)
retinitis pigmentosa 20Leber congenital amaurosis 2retinitis pigmentosa 87 with choroidal involvementRPE65-related recessive retinopathy
HGNC:10294UniProt:Q16518
SPATA7Spermatogenesis-associated protein 7Candidate gene tested inTolerante
FUNÇÃO

Involved in the maintenance of both rod and cone photoreceptor cells (By similarity). It is required for recruitment and proper localization of RPGRIP1 to the photoreceptor connecting cilium (CC), as well as photoreceptor-specific localization of proximal CC proteins at the distal CC (By similarity). Maintenance of protein localization at the photoreceptor-specific distal CC is essential for normal microtubule stability and to prevent photoreceptor degeneration (By similarity)

LOCALIZAÇÃO

Cytoplasm, cytoskeleton, cilium axonemeCytoplasm, cytoskeleton, cilium basal bodyCytoplasm, cytoskeletonCell projection, cilium, photoreceptor outer segment

MECANISMO DE DOENÇA

Leber congenital amaurosis 3

A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.

EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
61.8 TPM
Cérebro - Hemisfério cerebelar
16.1 TPM
Ovário
14.7 TPM
Pituitária
14.6 TPM
Cerebelo
13.8 TPM
OUTRAS DOENÇAS (4)
Leber congenital amaurosis 3severe early-childhood-onset retinal dystrophyLeber congenital amaurosisretinitis pigmentosa
HGNC:20423UniProt:Q9P0W8
LCA5LebercilinCandidate gene tested inTolerante
FUNÇÃO

Involved in intraflagellar protein (IFT) transport in photoreceptor cilia. Plays a role in the ciliary transport of photoreceptors outer segment proteins

LOCALIZAÇÃO

Cytoplasm, cytoskeletonCytoplasm, cytoskeleton, cilium axonemeCytoplasm, cytoskeleton, cilium basal bodyCytoplasm, cytoskeleton, microtubule organizing center, centrosomeCell projection, cilium

MECANISMO DE DOENÇA

Leber congenital amaurosis 5

A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.

EXPRESSÃO TECIDUAL(Ubíquo)
Cervix Ectocervix
8.4 TPM
Cervix Endocervix
8.0 TPM
Fallopian Tube
7.9 TPM
Útero
7.7 TPM
Ovário
7.4 TPM
OUTRAS DOENÇAS (3)
Leber congenital amaurosis 5severe early-childhood-onset retinal dystrophyLeber congenital amaurosis
HGNC:31923UniProt:Q86VQ0
LRATLecithin retinol acyltransferaseCandidate gene tested inTolerante
FUNÇÃO

Transfers the acyl group from the sn-1 position of phosphatidylcholine to all-trans retinol, producing all-trans retinyl esters (PubMed:9920938). Retinyl esters are storage forms of vitamin A (Probable). LRAT plays a critical role in vision (Probable). It provides the all-trans retinyl ester substrates for the isomerohydrolase which processes the esters into 11-cis-retinol in the retinal pigment epithelium; due to a membrane-associated alcohol dehydrogenase, 11 cis-retinol is oxidized and conver

LOCALIZAÇÃO

Endoplasmic reticulum membraneRough endoplasmic reticulumEndosome, multivesicular bodyCytoplasm, perinuclear region

VIAS BIOLÓGICAS (2)
The canonical retinoid cycle in rods (twilight vision)Retinoid metabolism and transport
MECANISMO DE DOENÇA

Leber congenital amaurosis 14

A severe dystrophy of the retina, typically becoming evident in the first years of life. Visual function is usually poor and often accompanied by nystagmus, sluggish or near-absent pupillary responses, photophobia, high hyperopia and keratoconus.

EXPRESSÃO TECIDUAL(Baixa expressão)
Testículo
3.3 TPM
Brain Spinal cord cervical c-1
3.1 TPM
Tireoide
2.8 TPM
Cólon sigmoide
2.4 TPM
Nervo tibial
2.2 TPM
OUTRAS DOENÇAS (4)
Leber congenital amaurosis 14severe early-childhood-onset retinal dystrophyretinitis pigmentosaLeber congenital amaurosis
HGNC:6685UniProt:O95237
PROM1Prominin-1Disease-causing germline mutation(s) inTolerante
FUNÇÃO

May play a role in cell differentiation, proliferation and apoptosis (PubMed:24556617). Binds cholesterol in cholesterol-containing plasma membrane microdomains and may play a role in the organization of the apical plasma membrane in epithelial cells. During early retinal development acts as a key regulator of disk morphogenesis. Involved in regulation of MAPK and Akt signaling pathways. In neuroblastoma cells suppresses cell differentiation such as neurite outgrowth in a RET-dependent manner (P

LOCALIZAÇÃO

Apical cell membraneCell projection, microvillus membraneCell projection, cilium, photoreceptor outer segmentEndoplasmic reticulumEndoplasmic reticulum-Golgi intermediate compartment

VIAS BIOLÓGICAS (1)
Developmental Lineage of Pancreatic Ductal Cells
MECANISMO DE DOENÇA

Retinitis pigmentosa 41

A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

EXPRESSÃO TECIDUAL(Ubíquo)
Glândula salivar
69.0 TPM
Rim - Medula
33.8 TPM
Cólon transverso
13.8 TPM
Intestino delgado
11.7 TPM
Testículo
8.3 TPM
OUTRAS DOENÇAS (7)
Stargardt disease 4retinitis pigmentosa 41retinal macular dystrophy type 2cone-rod dystrophy 12
HGNC:9454UniProt:O43490
CNGB3Cyclic nucleotide-gated channel beta-3Candidate gene tested inTolerante
FUNÇÃO

Pore-forming subunit of the cone cyclic nucleotide-gated channel. Mediates cone photoresponses at bright light converting transient changes in intracellular cGMP levels into electrical signals. In the dark, cGMP levels are high and keep the channel open enabling a steady inward current carried by Na(+) and Ca(2+) ions that leads to membrane depolarization and neurotransmitter release from synaptic terminals. Upon photon absorption cGMP levels decline leading to channel closure and membrane hyper

LOCALIZAÇÃO

Cell membrane

MECANISMO DE DOENÇA

Stargardt disease 1

An autosomal recessive form of Stargardt disease, a retinal degenerative disease characterized by macular dystrophy, progressive bilateral atrophy of the foveal retinal pigment epithelium, and accumulation of fluorescent flecks around the macula and/or in the central and near-peripheral areas of the retina. STGD1 patients typically lose central vision in their first or second decade of life.

OUTRAS DOENÇAS (4)
achromatopsia 3achromatopsiaStargardt diseasecone dystrophy
HGNC:2153UniProt:Q9NQW8
PRPH2Peripherin-2Candidate gene tested inTolerante
FUNÇÃO

Essential for retina photoreceptor outer segment disk morphogenesis, may also play a role with ROM1 in the maintenance of outer segment disk structure (By similarity). Required for the maintenance of retinal outer nuclear layer thickness (By similarity). Required for the correct development and organization of the photoreceptor inner segment (By similarity)

LOCALIZAÇÃO

MembraneCell projection, cilium, photoreceptor outer segmentPhotoreceptor inner segment

MECANISMO DE DOENÇA

Retinitis pigmentosa 7

A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

EXPRESSÃO TECIDUAL(Ubíquo)
Testículo
9.3 TPM
Ovário
6.8 TPM
Cervix Ectocervix
5.7 TPM
Pituitária
5.3 TPM
Músculo esquelético
5.0 TPM
OUTRAS DOENÇAS (13)
retinitis pigmentosa 7vitelliform macular dystrophy 3fundus albipunctatuschoroidal dystrophy, central areolar 2
HGNC:9942UniProt:P23942
ELOVL4Very long chain fatty acid elongase 4Disease-causing germline mutation(s) inModerado
FUNÇÃO

Catalyzes the first and rate-limiting reaction of the four reactions that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids (VLCFAs) per cycle. Condensing enzyme that catalyzes the synthesis of very long chain saturated (VLC-SFA) and polyunsaturated (PUFA) fatty acids that are involved in multiple biological processes as precursors of membrane lipids and

LOCALIZAÇÃO

Endoplasmic reticulum membrane

VIAS BIOLÓGICAS (1)
Synthesis of very long-chain fatty acyl-CoAs
MECANISMO DE DOENÇA

Stargardt disease 3

A form of Stargardt disease, a common hereditary macular degeneration characterized by decreased central vision, atrophy of the macula and underlying retinal pigment epithelium, and frequent presence of prominent flecks in the posterior pole of the retina. STGD3 is an autosomal dominant form with onset most commonly in the second decade of life.

EXPRESSÃO TECIDUAL(Ubíquo)
Skin Sun Exposed Lower leg
68.2 TPM
Skin Not Sun Exposed Suprapubic
64.1 TPM
Cérebro - Hemisfério cerebelar
43.9 TPM
Cerebelo
27.9 TPM
Brain Frontal Cortex BA9
23.3 TPM
OUTRAS DOENÇAS (4)
spinocerebellar ataxia type 34Stargardt disease 3congenital ichthyosis-intellectual disability-spastic quadriplegia syndromeStargardt disease
HGNC:14415UniProt:Q9GZR5

Medicamentos e terapias

EMIXUSTATPhase 3

Mecanismo: Retinoid isomerohydrolase inhibitor

METFORMINPhase 1

Mecanismo: Mitochondrial complex I (NADH dehydrogenase) inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

2,358 variantes patogênicas registradas no ClinVar.

🧬 ABCA4: NM_000350.3(ABCA4):c.968T>C (p.Leu323Pro) ()
🧬 ABCA4: NM_000350.3(ABCA4):c.2965G>T (p.Val989Phe) ()
🧬 ABCA4: NM_000350.3(ABCA4):c.5160del (p.Thr1721fs) ()
🧬 ABCA4: NM_000350.3(ABCA4):c.5116del (p.Arg1705_Val1706insTer) ()
🧬 ABCA4: NM_000350.3(ABCA4):c.176A>G (p.Lys59Arg) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 497 variantes classificadas pelo ClinVar.

447
50
Patogênica (89.9%)
VUS (10.1%)
VARIANTES MAIS SIGNIFICATIVAS
ABCA4: NM_000350.3(ABCA4):c.42C>A (p.Asn14Lys) [Pathogenic]
PROM1: NM_006017.3(PROM1):c.484_485del (p.Leu162fs) [Likely pathogenic]
RDH8: NM_015725.4(RDH8):c.262+1G>A [Pathogenic]
PROM1: NM_006017.3(PROM1):c.154del (p.Ile52fs) [Pathogenic/Likely pathogenic]
ABCA4: NM_000350.3(ABCA4):c.5329A>G (p.Met1777Val) [Conflicting classifications of pathogenicity]

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
3Fase 32
2Fase 26
1Fase 14
·Pré-clínico10
Medicamentos catalogadosEnsaios clínicos· 2 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Doença de Stargardt

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

20 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

NCT04545736 · Oral Metformin for Treatment of ABCA4 RetinopathyRecrutando
PHASE1, PHASE2
NCT02617966 · Rod and Cone Mediated Function in Retinal DiseaseRecrutando
NCT07425574 · A Study to Learn How Stargardt-type Eye Conditions Progress …Recrutando
NCT07439887 · Phase 1/2 Open-Label Dose-Escalation Study to Evaluate Safet…Recrutando
PHASE1, PHASE2
NCT07419334 · Study of ALK-001 on the Progression of Stargardt DiseaseRecrutando
PHASE3
NCT07266584 · Restoration of Central Vision With PRIMA in Patients With Ph…Recrutando
NA
NCT07502664 · Development and Evaluation of Functional Visual Field and Na…Recrutando
NCT06445322 · Prescreening Study to Identify Potential Stargardt Participa…Recrutando
NCT07161544 · A Study of AAVB-039 in Participants With Stargardt Disease (…Recrutando
PHASE1, PHASE2
NCT07417566 · A Study of DC6001 Tablet in Healthy Chinese Adult SubjectsRecrutando
PHASE1
NCT07002398 · Safety and Preliminary Efficacy of VG801 in Patients With AB…Recrutando
PHASE1, PHASE2
NCT06467344 · Study to Evaluate ACDN-01 in ABCA4-related Stargardt Retinop…Recrutando
PHASE1, PHASE2
NCT06319872 · The Effects of Disulfiram (Antabuse®) on Visual Acuity in Pa…Recrutando
PHASE1
NCT06435000 · An Observational Study in Subjects to Follow the Progression…Recrutando
NCT06591806 · An Observational Study in Children and Adults With Stargardt…Recrutando
NCT07063251 · An Clinical Study Evaluating the Safety, Tolerability, and e…Recrutando
EARLY_PHASE1
NCT06942572 · A Phase 1/2, First-in-Human Dose Escalation/Expansion Study …Recrutando
PHASE1, PHASE2
NCT02435940 · Inherited Retinal Degenerative Disease RegistryRecrutando
NCT06805474 · A Prospective Observational Study to Assess the Reliability …Recrutando
NCT03011541 · Stem Cell Ophthalmology Treatment Study IIRecrutando
NA

Outros ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
820 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 820

#1

Automated Quantification of Decreased Fundus Autofluorescence in Stargardt Disease Using Starguage: Validation against Manual Grading Standards.

Ophthalmology science2026 Apr

To evaluate the repeatability and reproducibility of Starguage, a novel automated method, compared with manual segmentation for measuring decreased autofluorescence (DAF) and definitely decreased autofluorescence (DDAF) in fundus autofluorescence (FAF) images of patients with Stargardt disease. A cross-sectional reproducibility and agreement study. A total of 316 eyes from 158 genetically confirmed Stargardt patients were analyzed. For intragrader repeatability, 114 FAF images were reassessed in a masked, repeated-measures design. Decreased autofluorescence and DDAF lesion areas were independently quantified by five certified graders using either manual delineation with Heidelberg RegionFinder or a threshold-based automated algorithm, with automated quantification and cross-method agreement analyses restricted to a prespecified central 6-mm fovea-centered region. Agreement and repeatability were assessed using intraclass correlation coefficients (ICCs), standard error of measurement (SEM), minimal detectable change (MDC), Lin's concordance correlation coefficient (CCC), Bland-Altman plots, and Passing-Bablok regression. Both raw and square-root-transformed lesion areas were evaluated. Repeatability (intragrader ICC, SEM, and MDC), reproducibility (intergrader ICC), and agreement (CCC and bias in regression analysis) between and within manual and automated methods. The automated method achieved excellent intragrader repeatability for both DAF and DDAF (ICCs ≥0.988, SEM ≤0.71 mm2, MDC ≤1.98 mm2), with minimal operator influence. Manual measurements showed variable repeatability (DAF ICCs 0.909-0.974; DDAF ICCs as low as 0.837), with square-root transformation reducing SEM and MDC. Intergrader reproducibility was highest for automated methods (ICC = 0.988-0.992), whereas manual methods ranged from 0.764-0.939 (raw) and 0.867-0.922 (transformed). Cross-method agreement was strong (CCC = 0.91-0.96), though minor proportional and constant bias was observed in raw DAF data. The automated approach provides near-perfect repeatability and high agreement with manual grading, offering a scalable, objective alternative for quantifying hypo-auto-fluorescent lesions in Stargardt disease. Manual methods are generally reliable but more variable, especially for DDAF, and benefit from square-root transformation. Findings reflect a pediatric/adolescent single-trial predominantly Asian cohort. The authors have no proprietary or commercial interest in any materials discussed in this article.

#2

Precise CRISPR/Cas9 and Cas12 Correction Using Lipoplexes in Retinal Models Derived from Patients with Inherited Retinal Dystrophies.

Cells2026 Mar 04

Gene editing, particularly CRISPR/Cas technology, represents a promising approach for the treatment of rare genetic diseases, including inherited retinal dystrophies, for which effective therapies are largely unavailable. Despite extensive research investigating gene editing across a wide range of cell types, transient delivery of CRISPR/Cas components and efficient homology-directed repair (HDR) in differentiated cells remain challenging. In this study, we employed hiPSCs derived from patients with Stargardt disease or Best disease, carrying pathogenic variants in ABCA4 or BEST1, respectively, to explore gene editing in human models. CRISPR/Cas9 and Cas12 nucleases were delivered into hiPS-derived retinal pigment epithelium (RPE) and retinal organoids using lipoplexes and compared with electroporation. We evaluated transfection efficiency, sgRNA-mediated DNA cleavage, and HDR-based correction. Precise repair of the pathogenic BEST1 variant was successfully achieved in hiPS-derived RPE cells using both nucleases, with Cas12 yielding the highest efficiency, exceeding 10% of HDR correction. Edited RPE cells preserved normal morphology and expressed specific maturity markers. In contrast, retinal organoids exhibited moderate transfection efficiency but showed no detectable CRISPR/Cas-induced DNA cleavage, highlighting the need for further optimization of gene editing in more complex cellular tissues. This study demonstrates, for the first time, precise correction of a single-nucleotide mutation in patient-derived RPE using CRISPR/Cas9 and Cas12 delivered using lipoplexes. These findings underscore the therapeutic potential of CRISPR/Cas-based strategies for inherited retinal dystrophies and provide a proof of concept for future clinical approximations.

#3

Longitudinal Changes of Fundus Autofluorescence and Correlation With Visual Acuity in ABCA4-Associated Stargardt Disease.

Investigative ophthalmology &amp; visual science2026 Mar 02

To assess cross-sectional and longitudinal relationships between fundus autofluorescence (FAF) and visual acuity (VA) in ABCA4-associated Stargardt disease (STGD1). Secondary analysis used data from 301 STGD1 participants (566 study eyes) enrolled in the international ProgStar studies. The study reading center graded the FAF images qualitatively and quantitatively, including areas of atrophy reflected as definitely decreased autofluorescence (DDAF) and questionably decreased autofluorescence (QDAF). Linear models estimated cross-sectional and longitudinal associations between the growth of DDAF area and total DAF area (i.e., sum of DDAF and QDAF areas) and change in VA. At the first visit, the median age was 30 years (range, 7-69); 55% were women. Mean ± SD VA was 0.73 ± 0.36 logMAR; mean total DAF area was 3.4 ± 3.8 mm2; and 42% of eyes had no DDAF. Cross-sectionally, increasing age and duration of symptoms, younger age of symptom onset, and larger QDAF area were significantly associated with worse VA (P < 0.05). Mean follow-up was 3.3 years (range, 0.5-10.3). There was no statistically significant relationship between growth of total DAF or DDAF areas and change in VA (P > 0.05). Characteristics other than DAF area (e.g., flecks presence) may influence VA. Some patients remained DDAF free longitudinally and thus would be excluded in STGD1 trials that use DDAF as the primary endpoint. The cross-sectional relationship between DAF and VA confirmed the association of VA with retinal pigment epithelium (RPE) structure. The lack of a significant association between growth in DAF areas with change in VA over follow-up points to challenges resulting from slow progression and large VA variability. Exploring outcome measures from other modalities is warranted for designing STGD1 trials.

#4

Advanced therapeutic approaches for inherited retinal diseases: an umbrella review.

BMJ open2026 Feb 27

To evaluate the efficacy and safety of advanced therapeutic approaches for inherited retinal disease (IRD) using evidence from systematic reviews and meta-analyses. Umbrella review. We searched for Epistemonikos, PubMed, Scopus, PsycInfo, Google Scholar, Joanna Briggs Institute Evidence Synthesis, the Cochrane Database of Systematic Reviews and Database of Abstracts of Reviews of Effects from inception to November 2024. This included English-language systematic review and meta-analysis assessing advanced therapies in patients with IRD (including congenital retinal dystrophies, retinal dystrophies, retinitis pigmentosa (RP), Stargardt disease, X linked RP, achromatopsia, cone-rod dystrophy, choroideraemia and X linked retinoschisis). Reviews that did not meet the methodological quality threshold were excluded. Two reviewers independently screened and extracted the data, with disagreements resolved by consensus. Findings were synthesised narratively due to the substantial overlap of primary studies. Six systematic reviews and meta-analyses published from 2020 onwards were included, comprising between 6 and 21 primary studies per review. The therapies evaluated included gene therapy, cell-based therapy and stem cell-based interventions. Reported effect estimates showed modest to clinically meaningful improvements in best-corrected visual acuity and retinal structural outcomes in selected IRD subtypes, although effect sizes varied widely across interventions and conditions. The GRADE certainty of evidence ranged from moderate to low, reflecting bias, imprecision and heterogeneity risks. Substantial overlap of primary studies was observed (corrected covered area = 28.9%), precluding quantitative pooling across reviews. The findings suggest notable improvements in visual acuity, retinal structure and other critical outcomes, with therapies such as cell therapy, gene therapy and stem cell therapy showing promising results in enhancing treatment efficacy. Although there are examples of successes with supportive evidence, the overall evidence is not sufficiently strong to make general recommendations, as studies still need to be evaluated on a case-by-case basis. Further high-quality, large-scale randomised controlled trials are needed to better confirm their efficacy and safety.

#5

Wnt signaling dysregulation drives Stargardt disease pathogenesis.

Science China. Life sciences2026 Feb 12

Stargardt disease, the most common inherited juvenile macular degeneration, is primarily caused by ABCA4 mutations, yet the cellular origin of pathology remains debated. Here, we identify a critical role for Wnt signaling dysregulation, specifically non-canonical Wnt pathway suppression, in driving retinal pigment epithelium (RPE) dysfunction as a central mechanism of disease. Using patient-derived iPSC-RPE harboring distinct ABCA4 mutations, we demonstrate that ABCA4 loss disrupts RPE hexagonal morphology, reduces pigmentation, and aberrantly activates neural retinal differentiation. Mechanistically, transcriptomic and functional analyses revealed pronounced downregulation of non-canonical Wnt signaling (e.g., Wnt5a), which correlated with epithelial disorganization and ectopic neurogenesis. Notably, while canonical Wnt activation failed to rescue these pathological defects, pharmacological stimulation of non-canonical Wnt signaling restored RPE integrity and suppressed neural transdifferentiation in a mutation-dependent manner. These findings not only redefine ABCA4 as a regulator of epithelial Wnt crosstalk but also unveil a divergence in Wnt pathway vulnerability among patients. Our work positions non-canonical Wnt agonism as a therapeutic strategy for Stargardt and underscores the broader imperative for mutation-tailored interventions in inherited retinal diseases. By revealing phenotypic convergence on Wnt signaling dysregulation in ABCA4-deficient RPE, this study provides a framework for understanding epithelial-pathway-driven degeneration, with implications for AMD, fibrosis, and developmental disorders characterized by polarity loss.

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📚 EuropePMC578 artigos no totalmostrando 197

2026

Crop-OCT: a Fully Integrated Imageomics Pipeline to Identify Regional and Focal Retinopathy in Murine Models.

bioRxiv : the preprint server for biology
2026

Retinal gene therapies for inherited ocular diseases: Translational delivery strategies from bench to bedside.

Journal of controlled release : official journal of the Controlled Release Society
2026

Nutritional supplements: current evidence for retinitis pigmentosa and Stargardt disease.

Current opinion in ophthalmology
2026

Automated Quantification of Decreased Fundus Autofluorescence in Stargardt Disease Using Starguage: Validation against Manual Grading Standards.

Ophthalmology science
2026

Phenotype-integrated reinterpretation of laboratory-reported ABCA4 gene sequencing results improves molecular diagnostic rate in Black/non-White patients and those with late-onset Stargardt macular dystrophy.

Ophthalmic genetics
2026

The Liver-Eye Axis of Dietary Vitamin A Homeostasis: A Review of Mechanisms, Receptors, and Visual Outcomes.

Nutrients
2026

Precise CRISPR/Cas9 and Cas12 Correction Using Lipoplexes in Retinal Models Derived from Patients with Inherited Retinal Dystrophies.

Cells
2026

Retinal vasoproliferative tumors in pediatric retinal dystrophies.

Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus
2026

Longitudinal Changes of Fundus Autofluorescence and Correlation With Visual Acuity in ABCA4-Associated Stargardt Disease.

Investigative ophthalmology &amp; visual science
2026

Advanced therapeutic approaches for inherited retinal diseases: an umbrella review.

BMJ open
2026

Acid sphingomyelinase inhibition restores RPE homeostasis and photoreceptor function in preclinical Stargardt macular degeneration models.

bioRxiv : the preprint server for biology
2026

Mental health outcomes in patients with inherited retinal diseases: a systematic review and meta-analysis.

International journal of retina and vitreous
2026

Wnt signaling dysregulation drives Stargardt disease pathogenesis.

Science China. Life sciences
2026

Genetic Correlates of Phenotypic Variability in c.5882G>A p.(Gly1961Glu)-Associated Stargardt Disease.

Investigative ophthalmology &amp; visual science
2026

Ferroptosis in retinal pigment epithelial cells: Current status and future developments.

Indian journal of ophthalmology
2026

More insights from Abca4-/- mouse models of recessive Stargardt disease.

The Journal of biological chemistry
2026

Mesopic microperimetry in Stargardt disease: Application and reliability.

Acta ophthalmologica
2026

[Clinical and genetic aspects of inherited retinal dystrophies : Phenotypic and molecular characterization of 1000 IRD patients in a German tertiary referral center].

Die Ophthalmologie
2026

A Comparison of Randomizing Either One Eye or Both Eyes in Clinical Trials for Stargardt Disease Type 1.

Ophthalmology science
2026

ABCA4-associated retinopathy complicated by didanosine-associated retinal toxicity.

American journal of ophthalmology case reports
2026

Effects of Gastric Acid Suppression, Cytochrome P4503A Inhibition and Induction, and Food on the Pharmacokinetics of Tinlarebant in Healthy Adults.

Clinical pharmacology in drug development
2026

Using RNA-targeting CRISPR-Cas13 and engineered U1 systems to target ABCA4 splice variants in Stargardt disease.

Molecular therapy. Nucleic acids
2025

Structure-Function Correlation Using a Targeted Image-Guided Microperimetry Approach for Retinal Atrophic Diseases: A Methods Study.

Translational vision science &amp; technology
2026

Homozygous initiation codon-altering complex variant causes rapid-onset chorioretinopathy phenotype in ABCA4 disease.

Documenta ophthalmologica. Advances in ophthalmology
2026

A novel modifier gene therapy to treat Stargardt disease: Phase 1 GARDian1 Trial Insights.

Eye (London, England)
2026

Inherited retinal disorders in Scotland: A 5 year assessment.

Eye (London, England)
2026

Benchmarking AlphaMissense against ClinVar for Diagnostic Interpretation of Missense Variants in Inherited Retinal Diseases.

Ophthalmology science
2025

Novel developments in retinal regeneration: Advances and future outlooks in stem cell therapy.

World journal of stem cells
2025

Mechanisms and Functions of Chromophore Regeneration in the Classical Visual Cycle: Implications for Retinal Disease Pathogenesis and Therapy.

Biomolecules
2026

Atypical Retinitis Pigmentosa With Macular Sparing in a Patient With Compound Heterozygous ABCA4 Variants: A Case Report and Diagnostic Challenge.

Clinical case reports
2025

Stem cell therapy for inherited retinal diseases: Trends and insights from 2000 to 2024.

Cell transplantation
2025

A self-supervised learning method for detection of retinitis pigmentosa and Stargardt disease.

Scientific reports
2025

Evaluating the clinical utility of multimodal large language models in rare maculopathy.

Scientific reports
2025

Discrete Wavelet Transform Analysis of PERG Signal Energies for Differentiating Retinal Pathologies.

Annual International Conference of the IEEE Engineering in Medicine and Biology Society. IEEE Engineering in Medicine and Biology Society. Annual International Conference
2025

Application of Machine Learning to Discriminate Photoreceptor Cell Species in Xenotransplanted Chimeric Retinas.

Clinical and translational science
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

Activation of GSDME by all-trans-retinal increases sensitivity to photoreceptor ferroptosis.

International journal of biological sciences
2025

Loss of retinal stem cell reserve and lipofuscin accumulation accelerates cone-rod degeneration and replicates Stargardt disease in abca4b null zebrafish.

Scientific reports
2025

Hyperreflective Choroidal Foci: A Comprehensive Review.

Journal of ophthalmic &amp; vision research
2026

Incidental Finding of Stargardt Disease in a Healthy Control.

JAMA ophthalmology
2026

COMPARING THE ABILITY OF GENETIC TESTING TO PROVIDE A DEFINITIVE DIAGNOSIS IN PATIENTS WITH PERIPHERAL AND MACULAR INHERITED RETINAL DISEASE.

Retina (Philadelphia, Pa.)
2025

Contribution of genetic test results to patient management in ophthalmology: results from a Turkish Stargardt disease cohort.

Turkish journal of medical sciences
2026

Large Choroidal Excavation in Stargardt Disease.

JAMA ophthalmology
2025

Dysregulated DNA Methylation in Abca4-/- Retinal Pigment Epithelium: Insights into Early Stage of Stargardt Disease.

International journal of molecular sciences
2025

Bisretinoids as a Source of Early Photoreceptor Pathology in Stargardt Disease.

Ophthalmic research
2025

Factors impacting experience of genetic testing among adults with inherited retinal diseases.

Journal of genetic counseling
2025

Ferrostatin-1, a ferroptosis inhibitor, mitigates all-trans-retinal-induced retinal pigment epithelium degeneration in mice.

Journal of translational medicine
2025

[Clinical and genetic analysis of a child with Stargardt disease type 1 caused by novel compound heterozygous variants of the ABCA4 gene].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2025

Early-Onset Stargardt Disease Caused by Homozygosity of a Complex ABCA4 Allele from Eastern Africa: Two Case Reports.

Case reports in ophthalmology
2025

ABCA4-mutant human retinal organoids sequencing reveals organoids application in inherited retinal diseases.

Experimental eye research
2025

Single-Cell Transcriptomics in Inherited Retinal Dystrophies: Current Findings and Emerging Perspectives.

Genes
2025

Decoding pediatric inherited retinal dystrophies: Bridging genetic complexity and clinical heterogeneity.

Progress in retinal and eye research
2025

Alterations in the foveal avascular zone and surrounding capillary network as important indicators of visual prognosis for hereditary macular dystrophy.

Taiwan journal of ophthalmology
2026

FACTORS INFLUENCING THE DELAYED DIAGNOSIS OF STARGARDT DISEASE AND IMPACT ON THERAPEUTIC OPPORTUNITIES.

Retina (Philadelphia, Pa.)
2025

Corrigendum to "Compendium of Clinical Variant Classification for 2,246 Unique ABCA4 Variants to Clarify Variant Pathogenicity in Stargardt Disease Using a Modified ACMG/AMP Framework".

Human mutation
2025

Stargardt's Connected Research Network Inaugural Meeting: Landscape Review and Horizon Scanning of Stargardt Disease.

Translational vision science &amp; technology
2025

Hyperreflective Outer Nuclear Layer as a Biomarker of Early Stargardt Disease. A Case Report.

Ceska a slovenska oftalmologie : casopis Ceske oftalmologicke spolecnosti a Slovenske oftalmologicke spolecnosti
2025

Subtle retinal degeneration in pigmented Abca4-/-Rdh8-/- mice.

Experimental eye research
2025

Cortical response to transient and long-term visual field loss.

Cerebral cortex (New York, N.Y. : 1991)
2025

Impact of Dietary Antioxidant Supplements on Atrophic Lesion Progression in Stargardt Disease: A Retrospective Observational Study.

Journal of ophthalmology
2025

Rescue of the Stargardt Disease Phenotype in Abca4 Knockout Mice Through Dietary Modulation of the Vitamin A Receptor RBPR2.

FASEB bioAdvances
2025

Bidirectional Hypoxic Extracellular Vesicle Signaling Between Müller Glia and Retinal Pigment Epithelium Regulates Retinal Metabolism and Barrier Function.

Biology
2025

Inherited retinal diseases in Kentucky: diagnostic yield, gene variants, and novel mutations in a U.S. population.

BMC medical genomics
2025

Lateral Geniculate Nucleus Volume Assessed by 7 Tesla MRI 3D MT-Weighted SILENT Protocol in Patients with STARGARDT Disease-Pilot Study.

Journal of clinical medicine
2025

Retinal Pigment Epithelium Transplantation in Retinal Disease: Clinical Trial Development, Challenges, and Future Directions.

Biomolecules
2025

Safety and efficacy of MCO-010 optogenetic therapy in patients with Stargardt disease in USA (STARLIGHT): an open-label multi-center Ph2 trial.

EClinicalMedicine
2025

Evaluating the Progression of Retinal Sensitivity Loss in Geographic Atrophy Using Machine-Learning-Based Structure-Function Correlation (OMEGA 2).

Investigative ophthalmology &amp; visual science
2025

Visual cycle and LC3-associated phagocytosis in retina: regulatory mechanisms and therapeutic potential.

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
2025

Flavoprotein Fluorescence Imaging in Stargardt Disease: Linking Metabolic Stress to Structural Damage.

Investigative ophthalmology &amp; visual science
2025

Rationally Designed, Short-Acting RPE65 Inhibitors for Visual Cycle-Associated Retinopathies.

Journal of medicinal chemistry
2025

Inactivation of cellular retinol-binding protein 1 protects against bis-retinoid accumulation and light-induced retinal degeneration in mice.

The Journal of biological chemistry
2025

Kidney-shaped maculopathy in Stargardt disease.

Journal francais d'ophtalmologie
2025

Advances in Precision Therapeutics and Gene Therapy Applications for Retinal Diseases: Impact and Future Directions.

Genes
2025

Stargardt's Disease: Molecular Pathogenesis and Current Therapeutic Landscape.

International journal of molecular sciences
2025

Ambispective study on the phenotypic progression of patients with Stargardt disease associated with ABCA4 gene mutations.

Archivos de la Sociedad Espanola de Oftalmologia
2025

Diagnostic Accuracy of AI Models in Detecting Different Inherited Retinal Diseases: A Systematic Review and Meta-Analysis.

Translational vision science &amp; technology
2025

The Visual Acuity Course in Stargardt Disease.

American journal of ophthalmology
2025

Fundus Autofluorescence in Inherited Retinal Disease: A Review.

Cells
2025

Nanovesicular Drug Delivery Systems for Rare Ocular Diseases: Advances, Challenges, and Future Directions.

AAPS PharmSciTech
2025

[Inherited retinal disorders: Current therapeutic options and future perspectives].

Klinische Monatsblatter fur Augenheilkunde
2025

Uncovering the Characteristics of Pupil Cycle Time (PCT) in Neuropathies and Retinopathies.

Vision (Basel, Switzerland)
2025

Advances in machine learning for ABCA4-related retinopathy: segmentation and phenotyping.

International ophthalmology
2025

Stargardt disease due to ABCA4 mutation in a young adult: Case report and current alternatives for optical and medical treatments.

Archivos de la Sociedad Espanola de Oftalmologia
2025

Structure-Function Correlation Using a Targeted Image- Guided Microperimetry Approach for Retinal Atrophic Diseases: A Methods Study.

medRxiv : the preprint server for health sciences
2025

ABCA4-associated disease in childhood and adolescence- a phenotype study.

Graefe's archive for clinical and experimental ophthalmology = Albrecht von Graefes Archiv fur klinische und experimentelle Ophthalmologie
2025

[Clinical manifestations and genetic variation analysis in six Chinese pedigrees affected with Stargardt disease].

Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics
2025

Identification and functional characterization of ABCA4 gene variants in three patients with Stargardt disease or retinitis pigmentosa.

Frontiers in genetics
2025

A deep learning model for diagnosis of inherited retinal diseases.

Scientific reports
2025

Stargardt Disease: Clinical Features and Genotypes in an Indian Cohort.

Clinical genetics
2025

Choroidal Hyperreflective Foci Represent a Common Finding Across Different Types of Macular Atrophy.

Investigative ophthalmology &amp; visual science
2025

Dark Adaptometry as a Diagnostic Tool in Retinal Diseases: Mechanisms and Clinical Utility.

Journal of clinical medicine
2025

Pathonign variants in recessive disorders: How extremely hypomorphic variants can be pathogenic and benign depending on the allele in trans.

Intractable &amp; rare diseases research
2025

Motion Processing in Visual Cortex of Maculopathy Patients.

The Journal of neuroscience : the official journal of the Society for Neuroscience
2025

Longitudinal scRNA-seq of retinal organoids derived from Stargardt disease patient with ABCA4 mutation.

Scientific data
2025

Stem cell therapy as treatment for Stargardt disease.

Therapeutic advances in ophthalmology
2025

Photorefractive keratectomy in a patient with Stargardt disease: Case report.

SAGE open medical case reports
2025

OCT Findings in a Patient with Mild ABCA4 Stargardt Disease.

Ophthalmology. Retina
2025

Rekindling Vision: Innovative Strategies for Treating Retinal Degeneration.

International journal of molecular sciences
2025

Multimodal imaging in autosomal recessive Stargardt's disease.

BMJ case reports
2025

Ethnic disparities in inherited retinal degenerations.

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie
2025

High Resolution Imaging and Fixation Analysis of Eccentric Preferred Retinal Loci in Macular Diseases.

Investigative ophthalmology &amp; visual science
2025

Progression of Atrophy as a Function of ABCA4 Variants and Age of Onset in Stargardt Disease.

Investigative ophthalmology &amp; visual science
2025

Advances and therapeutic opportunities in visual cycle modulation.

Progress in retinal and eye research
2025

Generation of a compound heterozygous ABCA4 rat model with pathological features of STGD1.

Human molecular genetics
2025

Clinical and Genetic Characteristics of 18 Patients from Southeast China with ABCA4-Associated Stargardt Disease.

International journal of molecular sciences
2025

Spontaneous release of an epiretinal membrane in an adult patient with Stargardt disease - A case report.

Retinal cases &amp; brief reports
2025

Founder Variants in the Mexican Population: A Systematic Review.

Archives of medical research
2025

Multidimensional Functional Phenotyping Based on Photoreceptor-Directed Temporal Contrast Sensitivity Defects in Inherited Retinal Diseases.

Investigative ophthalmology &amp; visual science
2025

Delivery of Human iPSC-Derived RPE Cells in Healthy Minipig Retina Results in Interaction Between Photoreceptors and Transplanted Cells.

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

Retinal gene therapy for Stargardt disease with dual AAV intein vectors is both safe and effective in large animal models.

Science advances
2025

[Pharmacotherapy of ABCA4-associated Retinal Dystrophies].

Klinische Monatsblatter fur Augenheilkunde
2025

Inherited retinal degeneration in Malay and Indian populations of Singapore and Malaysia: a prospective multicentre study.

Ophthalmic genetics
2025

Health state utilities associated with X-linked retinitis pigmentosa (XLRP).

The European journal of health economics : HEPAC : health economics in prevention and care
2025

Quantifying the Progression of Stargardt Disease in Double-Null ABCA4 Carriers Using Fundus Autofluorescence Imaging.

Translational vision science &amp; technology
2025

Rescue of the disease-associated phenotype in CRISPR-corrected hiPSCs as a therapeutic approach for inherited retinal dystrophies.

Molecular therapy. Nucleic acids
2025

Carrier Frequency of Autosomal Recessive Diseases in a Population Attending a Human Fertility Institute in Colombia.

JBRA assisted reproduction
2025

Retinal Detachment Accompanied by a Macular Hole in a Patient With ABCA4 and BEST1 Genetic Mutations.

Cureus
2025

Dark choroid sign in Stargardt disease.

Eye (London, England)
2025

Retinal thickness and visual acuity in early-onset Stargardt disease follow a non-linear progression curve: implications for clinical trials.

Ophthalmic genetics
2025

Pediatric Eye Screening: Current Standards and Gaps in Care.

Ophthalmic surgery, lasers &amp; imaging retina
2025

Intersection of Stargardt Dystrophy and AIDS: A Case Report.

Cureus
2025

Unravelling genotype-phenotype correlations in Stargardt disease using patient-derived retinal organoids.

Cell death &amp; disease
2025

WDR19-associated retinopathy presenting with adult-onset Stargardt-like phenotype.

Ophthalmic genetics
2025

Improving personalised genetic counselling for ABCA4 -associated retinopathy: Updated recurrence risk estimates.

Medizinische Genetik : Mitteilungsblatt des Berufsverbandes Medizinische Genetik e.V
2025

Proposing Zebrafish as a Model for Stargardt Disease.

Advances in experimental medicine and biology
2025

Frequency and Pattern of Gene Therapy Clinical Trials for Inherited Retinal Diseases.

Advances in experimental medicine and biology
2025

Assessment of ABCA4 Genetic Variants: Current Landscape and Future Prospects.

Advances in experimental medicine and biology
2025

ABCA4 c.5461-6T>C Causes Stargardt Disease Through Exon Skipping.

Advances in experimental medicine and biology
2025

Looking outside the box with a pathology aware AI approach for analyzing OCT retinal images in Stargardt disease.

Scientific reports
2025

Superotemporal predisposition to traumatic subretinal fibrosis in Stargardt disease: A case report.

American journal of ophthalmology case reports
2025

ABCA4 Deep Intronic Variants Contributed to Nearly Half of Unsolved Stargardt Cases With a Milder Phenotype.

Investigative ophthalmology &amp; visual science
2025

Clinical perspective on pluripotent stem cells derived cell therapies for the treatment of neurodegenerative diseases.

Advanced drug delivery reviews
2025

Revealing Molecular Diagnosis With Whole Exome Sequencing in Patients With Inherited Retinal Disorders.

Clinical genetics
2025

Advancing Insights into Pediatric Macular Diseases: A Comprehensive Review.

Journal of clinical medicine
2025

Genotype-phenotype correlations for 17 Chinese families with inherited retinal dystrophies due to homozygous variants.

Scientific reports
2025

Preclinical assessment of splicing modulation therapy for ABCA4 variant c.768G>T in Stargardt disease.

Communications medicine
2025

Differential Diagnosis of Age-Related Macular Degeneration.

Klinische Monatsblatter fur Augenheilkunde
2025

Modifiers and their impact on inherited retinal diseases: a review.

Ophthalmic genetics
2025

High-efficiency base editing in the retina in primates and human tissues.

Nature medicine
2024

Quercetin Alleviates All-Trans-Retinal-Induced Photoreceptor Apoptosis and Retinal Degeneration by Inhibiting the ER Stress-Related PERK Signaling.

International journal of molecular sciences
2024

Rescue of Aberrant Splicing Caused by a Novel Complex Deep-intronic ABCA4 Allele.

Genes
2025

12-year cumulative incidence rate of rare retinal diseases: a nationwide study in Korea.

Eye (London, England)
2025

Heart-retina time analysis using electrocardiogram-coupled time-resolved dynamic optical coherence tomography.

Scientific reports
2024

Maculopathies: A Systematic Literature Review on Pathophysiology, Public Health, and Treatment.

Cureus
2024

Interruption of the visual cycle in a novel animal model induces progressive vision loss resembling Stargardts Disease.

Scientific reports
2025

CHOROIDAL HYPERREFLECTIVE FOCI AS BIOMARKERS OF SEVERITY IN STARGARDT DISEASE.

Retina (Philadelphia, Pa.)
2025

Inhibition of JNK signaling attenuates photoreceptor ferroptosis caused by all-trans-retinal.

Free radical biology &amp; medicine
2024

Evaluation of mesenchymal stem cells as an in vitro model for inherited retinal diseases.

Frontiers in cell and developmental biology
2025

Abca4, mutated in Stargardt disease, is required for structural integrity of cone outer segments.

Disease models &amp; mechanisms
2024

Characterization of the Subclinical Perilesional Zone in the Macula of Early-Stage ABCA4 Disease.

medRxiv : the preprint server for health sciences
2025

Distinguishing ABCA4 from PRPH2-related disease: qualitative analysis of examination and imaging features.

Ophthalmic genetics
2024

Identification of a novel mutation in the PROM1 gene associated with autosomal dominant Stargardt disease.

Asian journal of surgery
2025

PRPH2-ASSOCIATED RETINAL DISEASES: A SYSTEMATIC REVIEW OF PHENOTYPIC FINDINGS.

American journal of ophthalmology
2024

Lipopeptide-mediated Cas9 RNP delivery: A promising broad therapeutic strategy for safely removing deep-intronic variants in ABCA4.

Molecular therapy. Nucleic acids
2024

A comparative study of choroidal structural features in eyes with central macular atrophy related to Stargardt disease and non-exudative age-related macular degeneration.

Indian journal of ophthalmology
2024

Strabismus in an Adolescent With Stargardt Disease: An Atypical Presentation.

Cureus
2024

Adaptive optics scanning laser ophthalmoscopy in a heterogenous cohort with Stargardt disease.

Scientific reports
2024

Censoring the Floor Effect in Long-Term Stargardt Disease Microperimetry Data Produces a Faster Rate of Decline.

Ophthalmology science
2024

An uncommon case of retinitis pigmentosa patients based on clinical and genetic study.

The Medical journal of Malaysia
2024

Emerging Therapeutic Approaches and Genetic Insights in Stargardt Disease: A Comprehensive Review.

International journal of molecular sciences
2025

Progression Rate of Macular Retinal Pigment Epithelium Atrophy in Geographic Atrophy and Selected Inherited Retinal Dystrophies. A Systematic Review and Meta-Analysis.

American journal of ophthalmology
2024

Longitudinal imaging of 8-year progression in a teenager with Stargardt disease.

Asia-Pacific journal of ophthalmology (Philadelphia, Pa.)
2024

Presentation and Clinical Features of Stargardt Disease in a Series of Nigerian Patients.

Annals of African medicine
2024

Structural and functional characterization of the nucleotide-binding domains of ABCA4 and their role in Stargardt disease.

The Journal of biological chemistry
2024

Aberrant lipid accumulation and retinal pigment epithelium dysfunction in PRCD-deficient mice.

Experimental eye research
2024

The Surviving, Not Thriving, Photoreceptors in Patients with ABCA4 Stargardt Disease.

Diagnostics (Basel, Switzerland)
2024

The ABCs of Stargardt disease: the latest advances in precision medicine.

Cell &amp; bioscience
2024

Novel Variants in ABCA4-Related Retinopathies with Structural Re-Assessment of Variants of Uncertain Significance.

Ophthalmologica. Journal international d'ophtalmologie. International journal of ophthalmology. Zeitschrift fur Augenheilkunde
2024

Safety of intravitreally delivered AAV2 vector-mediated multi-characteristic opsin genetic construct in wild type beagle dogs.

The journal of gene medicine
2024

The Progression of Stargardt Disease as Determined by Spectral-Domain Optical Coherence Tomography over a 24-Month Period (ProgStar Report No. 18).

Ophthalmic research
2024

Multimodal in-vivo maps as a tool to characterize retinal structural biomarkers for progression in adult-onset Stargardt disease.

Frontiers in ophthalmology
2024

Clinical exome analysis and targeted gene repair of the c.1354dupT variant in iPSC lines from patients with PROM1-related retinopathies exhibiting diverse phenotypes.

Stem cell research &amp; therapy
2024

Recent advancements and applications of ophthalmic gene therapy strategies: A breakthrough in ocular therapeutics.

Experimental eye research
2024

Genetic Characterization of 191 Probands with Inherited Retinal Dystrophy by Targeted NGS Analysis.

Genes
2024

Novel and Recurrent Copy Number Variants in ABCA4-Associated Retinopathy.

International journal of molecular sciences
2024

Generation and characterization of a Stargardt's disease-specific induced pluripotent stem cell line (LVPEIi008-A) with a homozygous nonsense mutation in exon 44 of ABCA4.

Stem cell research
2024

Impaired cathepsin D in retinal pigment epithelium cells mediates Stargardt disease pathogenesis.

FASEB journal : official publication of the Federation of American Societies for Experimental Biology
2024

Time-Resolved Dynamic Optical Coherence Tomography for Retinal Blood Flow Analysis.

Investigative ophthalmology &amp; visual science
2024

Clinician-Driven Reanalysis of Exome Sequencing Data From Patients With Inherited Retinal Diseases.

JAMA network open
2024

Antisense Oligonucleotide-Based Rescue of Complex Intronic Splicing Defects in ABCA4.

Nucleic acid therapeutics
2024

Retinoic acid related orphan receptor α is a genetic modifier that rescues retinal degeneration in a mouse model of Stargardt disease and Dry AMD.

Gene therapy
2024

Nationwide Prevalence of Inherited Retinal Diseases in the Israeli Population.

JAMA ophthalmology
2024

ABCA4-related retinopathies in Lebanon.

Heliyon
2024

Artificial Intelligence (AI) for Early Diagnosis of Retinal Diseases.

Medicina (Kaunas, Lithuania)
2024

A Proximity Complementation Assay to Identify Small Molecules That Enhance the Traffic of ABCA4 Misfolding Variants.

International journal of molecular sciences
2024

Metabolomics facilitates differential diagnosis in common inherited retinal degenerations by exploring their profiles of serum metabolites.

Nature communications
2024

Generation of two induced pluripotent stem cell lines (LVPEIi007-B, LVPEIi008-B) from patients harboring homozygous mutation in ABCA4 (c.6088C>T) using non-integrative Sendai virus-based approach.

Stem cell research
2024

Preclinical Development of Antisense Oligonucleotides to Rescue Aberrant Splicing Caused by an Ultrarare ABCA4 Variant in a Child with Early-Onset Stargardt Disease.

Cells
2024

Representation of Women Among Individuals With Mild Variants in ABCA4-Associated Retinopathy: A Meta-Analysis.

JAMA ophthalmology
2024

Nationwide epidemiologic survey on incidence of macular dystrophy in Japan.

Japanese journal of ophthalmology
2024

LONGITUDINAL ADAPTIVE OPTICS SCANNING LASER OPHTHALMOSCOPY REVEALS REGIONAL VARIATION IN CONE AND ROD PHOTORECEPTOR LOSS IN STARGARDT DISEASE.

Retina (Philadelphia, Pa.)
2024

Understanding and Rescuing the Splicing Defect Caused by the Frequent ABCA4 Variant c.4253+43G>A Underlying Stargardt Disease.

Nucleic acid therapeutics
2024

Intravitreal Delivery of PEGylated-ECO Plasmid DNA Nanoparticles for Gene Therapy of Stargardt Disease.

Pharmaceutical research
2025

THE PERIPAPILLARY RETINA: A COMMON JUNCTURE IN STARGARDT DISEASE AND IDIOPATHIC INTRACRANIAL HYPERTENSION.

Retinal cases &amp; brief reports
2024

Automated identification of fleck lesions in Stargardt disease using deep learning enhances lesion detection sensitivity and enables morphometric analysis of flecks.

The British journal of ophthalmology
2024

Recurrent and Concurrent Prediction of Longitudinal Progression of Stargardt Atrophy and Geographic Atrophy.

medRxiv : the preprint server for health sciences
2024

ABCA4 variant screening in a Turkish cohort with Stargardt disease.

Ophthalmic genetics
Ver todos os 578 no EuropePMC

Associações

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Comunidades

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

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. Automated Quantification of Decreased Fundus Autofluorescence in Stargardt Disease Using Starguage: Validation against Manual Grading Standards.
    Ophthalmology science· 2026· PMID 41847073mais citado
  2. Precise CRISPR/Cas9 and Cas12 Correction Using Lipoplexes in Retinal Models Derived from Patients with Inherited Retinal Dystrophies.
    Cells· 2026· PMID 41827889mais citado
  3. Longitudinal Changes of Fundus Autofluorescence and Correlation With Visual Acuity in ABCA4-Associated Stargardt Disease.
    Investigative ophthalmology &amp; visual science· 2026· PMID 41773771mais citado
  4. Advanced therapeutic approaches for inherited retinal diseases: an umbrella review.
    BMJ open· 2026· PMID 41760155mais citado
  5. Wnt signaling dysregulation drives Stargardt disease pathogenesis.
    Science China. Life sciences· 2026· PMID 41701438mais citado
  6. Uncovering mitochondrial defects in photoreceptors opens therapeutic opportunities for Stargardt disease.
    Proc Natl Acad Sci U S A· 2026· PMID 41984830recente
  7. Integrated transcriptomic and metabolomic analyses reveal distinct energy metabolic signatures and functional properties of RPE cells under two culture conditions.
    Sci Rep· 2026· PMID 41963383recente
  8. Crop-OCT: a Fully Integrated Imageomics Pipeline to Identify Regional and Focal Retinopathy in Murine Models.
    bioRxiv· 2026· PMID 41867838recente
  9. Retinal gene therapies for inherited ocular diseases: Translational delivery strategies from bench to bedside.
    J Control Release· 2026· PMID 41865892recente
  10. Nutritional supplements: current evidence for retinitis pigmentosa and Stargardt disease.
    Curr Opin Ophthalmol· 2026· PMID 41861379recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:827(Orphanet)
  2. MONDO:0019353(MONDO)
  3. GARD:181(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Artigo Wikipedia(Wikipedia)

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

Doença de Stargardt
Compêndio · Raras BR

Doença de Stargardt

ORPHA:827 · MONDO:0019353
Prevalência
1-5 / 10 000
Herança
Autosomal dominant, Autosomal recessive
CID-10
H35.5 · Distrofias hereditárias da retina
CID-11
Ensaios
32 ativos
Medicamentos
2 registrados
Início
Adolescent, Adult, Childhood, Elderly
Prevalência
13.0 (Europe)
MedGen
UMLS
C0271093
EuropePMC
Wikipedia
Papers 10a
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