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Charcot-Marie-Tooth ligada ao X
ORPHA:64747CID-10 · G60.0CID-11 · 8C20.0DOENÇA RARA

Subtipo da doença de Charcot-Marie-Tooth com defeitos genéticos no cromossomo X.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Subtipo da doença de Charcot-Marie-Tooth com defeitos genéticos no cromossomo X.

Pesquisas ativas
1 ensaio
1 total registrados no ClinicalTrials.gov
Publicações científicas
209 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 100 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
1.6
Europe
Herança
X-linked dominant
+1
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: G60.0
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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

🧠
Neurológico
20 sintomas
💪
Músculos
16 sintomas
🦴
Ossos e articulações
10 sintomas
👁️
Olhos
5 sintomas
🫃
Digestivo
2 sintomas
👂
Ouvidos
2 sintomas

+ 42 sintomas em outras categorias

Características mais comuns

Pé valgo
Atrofia do músculo esquelético
Velocidade de condução nervosa anormal
Cifose
Nível diminuído de fosforribosilpirofosfato sintetase
EMG: sinais de desnervação crônica
97sintomas
Sem dados (97)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 97 características clínicas mais associadas, ordenadas por frequência.

Pé valgoPes valgus
Atrofia do músculo esqueléticoSkeletal muscle atrophy
Velocidade de condução nervosa anormalAbnormal nerve conduction velocity
CifoseKyphosis
Nível diminuído de fosforribosilpirofosfato sintetaseDecreased phosphoribosylpyrophosphate synthetase level

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico209PubMed
Últimos 10 anos80publicações
Pico201812 papers
Linha do tempo
2026Hoje · 2026🧪 2010Primeiro ensaio clínico📈 2018Ano 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

4 genes identificados com associação a esta condição. Padrão de herança: X-linked dominant, X-linked recessive.

PRPS1Ribose-phosphate pyrophosphokinase 1Disease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Catalyzes the synthesis of phosphoribosylpyrophosphate (PRPP) that is essential for nucleotide synthesis

LOCALIZAÇÃO

VIAS BIOLÓGICAS (1)
5-Phosphoribose 1-diphosphate biosynthesis
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
78.0 TPM
Cérebro - Hemisfério cerebelar
64.3 TPM
Fibroblastos
58.6 TPM
Cerebelo
51.2 TPM
Tireoide
51.1 TPM
OUTRAS DOENÇAS (8)
phosphoribosylpyrophosphate synthetase superactivityCharcot-Marie-Tooth disease X-linked recessive 5Arts syndromehearing loss, X-linked 1
HGNC:9462UniProt:P60891
PDK3[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 3, mitochondrialDisease-causing germline mutation(s) inRestrito
FUNÇÃO

Inhibits pyruvate dehydrogenase activity by phosphorylation of the E1 subunit PDHA1, and thereby regulates glucose metabolism and aerobic respiration. Can also phosphorylate PDHA2. Decreases glucose utilization and increases fat metabolism in response to prolonged fasting, and as adaptation to a high-fat diet. Plays a role in glucose homeostasis and in maintaining normal blood glucose levels in function of nutrient levels and under starvation. Plays a role in the generation of reactive oxygen sp

LOCALIZAÇÃO

Mitochondrion matrix

VIAS BIOLÓGICAS (2)
Signaling by Retinoic AcidRegulation of pyruvate dehydrogenase (PDH) complex
MECANISMO DE DOENÇA

Charcot-Marie-Tooth disease, X-linked dominant, 6

A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy.

EXPRESSÃO TECIDUAL(Ubíquo)
Artéria tibial
33.5 TPM
Linfócitos
32.3 TPM
Aorta
27.0 TPM
Testículo
19.6 TPM
Sangue
16.1 TPM
OUTRAS DOENÇAS (1)
Charcot-Marie-Tooth disease X-linked dominant 6
HGNC:8811UniProt:Q15120
AIFM1Apoptosis-inducing factor 1, mitochondrialDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

Functions both as NADH oxidoreductase and as regulator of apoptosis (PubMed:17094969, PubMed:20362274, PubMed:23217327, PubMed:33168626). In response to apoptotic stimuli, it is released from the mitochondrion intermembrane space into the cytosol and to the nucleus, where it functions as a proapoptotic factor in a caspase-independent pathway (PubMed:20362274). Release into the cytoplasm is mediated upon binding to poly-ADP-ribose chains (By similarity). The soluble form (AIFsol) found in the nuc

LOCALIZAÇÃO

Mitochondrion intermembrane spaceMitochondrion inner membraneCytoplasmNucleusCytoplasm, perinuclear regionMitochondrionCytoplasm, cytosol

MECANISMO DE DOENÇA

Combined oxidative phosphorylation deficiency 6

A mitochondrial disease resulting in a neurodegenerative disorder characterized by psychomotor delay, hypotonia, areflexia, muscle weakness and wasting. Some patients manifest prenatal ventriculomegaly and severe postnatal encephalomyopathy.

OUTRAS DOENÇAS (4)
X-linked hereditary sensory and autonomic neuropathy with hearing lossCharcot-Marie-Tooth disease X-linked recessive 4spondyloepimetaphyseal dysplasia, Bieganski typesevere X-linked mitochondrial encephalomyopathy
HGNC:8768UniProt:O95831
GJB1Gap junction beta-1 proteinDisease-causing germline mutation(s) inAltamente restrito
FUNÇÃO

One gap junction consists of a cluster of closely packed pairs of transmembrane channels, the connexons, through which materials of low MW diffuse from one cell to a neighboring cell

LOCALIZAÇÃO

Cell membraneCell junction, gap junction

VIAS BIOLÓGICAS (3)
Oligomerization of connexins into connexonsGap junction assemblyTransport of connexins along the secretory pathway
MECANISMO DE DOENÇA

Charcot-Marie-Tooth disease, X-linked dominant, 1

A form of Charcot-Marie-Tooth disease, a disorder of the peripheral nervous system, characterized by progressive weakness and atrophy, initially of the peroneal muscles and later of the distal muscles of the arms. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathies characterized by severely reduced motor nerve conduction velocities (NCVs) (less than 38m/s) and segmental demyelination and remyelination, and primary peripheral axonal neuropathies characterized by normal or mildly reduced NCVs and chronic axonal degeneration and regeneration on nerve biopsy. CMTX1 has both demyelinating and axonal features. Central nervous system involvement may occur.

EXPRESSÃO TECIDUAL(Tecido-específico)
Fígado
187.2 TPM
Brain Spinal cord cervical c-1
172.2 TPM
Pâncreas
56.1 TPM
Substância negra
44.4 TPM
Cólon transverso
27.3 TPM
OUTRAS DOENÇAS (2)
Charcot-Marie-Tooth disease X-linked dominant 1X-linked progressive cerebellar ataxia
HGNC:4283UniProt:P08034

Variantes genéticas (ClinVar)

719 variantes patogênicas registradas no ClinVar.

🧬 PRPS1: NM_002764.4(PRPS1):c.155A>C (p.Asp52Ala) ()
🧬 PRPS1: NM_002764.4(PRPS1):c.878C>T (p.Ser293Phe) ()
🧬 PRPS1: NM_002764.4(PRPS1):c.655G>A (p.Val219Met) ()
🧬 PRPS1: NM_002764.4(PRPS1):c.851G>T (p.Cys284Phe) ()
🧬 PRPS1: GRCh37/hg19 Xq13.1-27.1(chrX:71017904-140066710)x4 ()
Ver todas no ClinVar

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

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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.
·Pré-clínico1
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 1 ensaio
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Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Charcot-Marie-Tooth ligada ao X

🗺️

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

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Publicações mais relevantes

Timeline de publicações
79 papers (10 anos)
#1

Case Report: Recurrent stroke-like episodes triggered by high-altitude exposure in X-linked charcot-marie-tooth disease.

Frontiers in genetics2026

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is a rare inherited neuropathy caused by mutations in the GJB1 gene, leading to progressive distal muscle weakness and atrophy. In this case study, a 37-year-old man presented with recurrent episodes of numbness involving the lips, right hand, and foot, followed by right-sided limb weakness and dysarthria four times over the past 20 years. Notably, the last three episodes were consistently triggered within 3 days after descent to sea level following exposure to high-altitude environments (8,500-10,000 feet, with transit above 13,000 feet). Based on the neurologic examination, brain magnetic resonance imaging (MRI), and genetic testing, the patient was diagnosed with X-linked Charcot-Marie-Tooth disease. This case underscores the importance of considering high-altitude exposure as a potential trigger and highlights the value of GJB1 testing in young patients presenting with acute stroke-like episodes and signs of peripheral neuropathy.

#2

Patient-derived neural organoids reveal developmental impairments associated with a novel GJB1 mutation in X-linked Charcot-Marie-Tooth disease.

Neurobiology of disease2026 Mar

Charcot-Marie-Tooth disease (CMT) is one of the most prevalent inherited peripheral neuropathies. CMT type X1 (CMTX1), caused by mutations in the GJB1 gene, represents the most common X-linked subtype with central nervous system (CNS) involvement. Here, we report the identification and functional characterization of a novel GJB1 variant (c.554C > T, p.Thr185Ile) in a CMTX1-affected family and its pathogenic impact using patient-derived induced pluripotent stem cells (iPSCs) and three-dimensional (3D) neural organoid models. The GJB1 gene encodes connexin 32 (Cx32), a gap junction protein. Immunofluorescent analysis revealed aberrant intracellular reduction and aggregation of the mutant Cx32 protein, suggesting impaired gap junction function. iPSC-derived neural organoids carrying the GJB1 mutation exhibited significant delay in neural differentiation and disrupted neural rosette organization. These findings underscore the critical role of Cx32 in neural development and provide a physiologically relevant platform for underlying CMTX1 pathological mechanisms on central nervous system. The established GJB1-variant organoid model holds promise for investigating genotype-phenotype correlations and facilitating the development of targeted therapeutic strategies for CMTX1.

#3

Facing the challenge of effective dosing, safety, and timing of intrathecal gene therapy for neurological disorders.

EBioMedicine2026 Jan
#4

[A case of X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) diagnosed based on recurrent brain lesions despite peripheral neuropathy responsive to immunotherapy].

Rinsho shinkeigaku = Clinical neurology2025 Oct 22

The patient is a 17-year-old male. He had a history of hospitalization for influenza at the age of 11, and Brain MRI at that time showed reversible brain lesions in the splenium of the corpus callosum and cerebral white matter. Fifteen months ago, he visited the pediatrics department due to dysphagia, dysarthria, facial paralysis, and muscle weakness. Brain MRI revealed lesions similar to those observed here, and nerve conduction study revealed demyelinating neuropathy. He was treated with intravenous immunoglobulin (IVIg) and intravenous methylprednisolone, and his symptoms disappeared within a few days and Brain MRI 5 weeks after treatment revealed that the lesions had disappeared. Three months ago, while walking, the patient developed a knee strain, which was thought to be a recurrence of the immune-mediated neuropathy. His subjective symptom disappeared after administration of IVIg. The patient was diagnosed with X-linked Charcot-Marie-Tooth disease (CMTX1) based on genetic testing, which revealed a pathological variant of GJB1, c.124A>T (p.Ser42Cys). Peripheral neuropathy in CMTX1 may present with fluctuating symptoms and can be responsive to IVIg treatment. Recurrent brain lesions should also be considered in the diagnosis of CMTX1.

#5

Random X chromosome inactivation in female Charcot-Marie-Tooth disease type X1: insights from sural nerve biopsy analysis.

BMC neurology2025 Aug 04

X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) is a hereditary neuropathy caused by mutations in the GJB1 gene encoding Connexin 32 (Cx32). Despite its X-linked dominant inheritance, it has been suggested that the variable phenotypic expression of the disease in females may be due to skewed X chromosome inactivation (XCI) in Schwann cells. This pilot study aimed to examine the XCI patterns in archived sural nerve biopsies of female patients diagnosed with CMTX1 for the first time in the literature. Two unrelated female CMTX1 patients, initially misdiagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP), with motor conduction blocks were included. GJB1 mutations were identified using whole-exome sequencing (WES). XCI patterns were analyzed using the human androgen receptor (HUMARA) assay on archived fresh-frozen sural nerve biopsy samples performed prior the genetic diagnosis and compared with age-matched vasculitic neuropathy controls. The GJB1 c.379 A > C mutation was identified in both CMTX1 patients. XCI analysis revealed a random XCI pattern in affected nerve tissues, with no significant differences between patients and controls. Contrary to previous hypotheses, our findings suggest that XCI does not contribute to phenotypic variability in female CMTX1 patients. These results highlight the complexity of CMTX1 pathophysiology and highlight the need for further studies on alternative mechanisms regulating disease severity in females.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC136 artigos no totalmostrando 80

2026

Case Report: Recurrent stroke-like episodes triggered by high-altitude exposure in X-linked charcot-marie-tooth disease.

Frontiers in genetics
2026

Patient-derived neural organoids reveal developmental impairments associated with a novel GJB1 mutation in X-linked Charcot-Marie-Tooth disease.

Neurobiology of disease
2026

Facing the challenge of effective dosing, safety, and timing of intrathecal gene therapy for neurological disorders.

EBioMedicine
2025

[A case of X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) diagnosed based on recurrent brain lesions despite peripheral neuropathy responsive to immunotherapy].

Rinsho shinkeigaku = Clinical neurology
2025

A Family With X-Linked Charcot-Marie-Tooth Disease Type 1: A Case for Reclassifying a Variant of Uncertain Significance in GJB1and Review of the Literature.

Journal of clinical neuromuscular disease
2025

Random X chromosome inactivation in female Charcot-Marie-Tooth disease type X1: insights from sural nerve biopsy analysis.

BMC neurology
2025

A dose escalation and safety study of AAVrh10-mediated Schwann cell-targeted gene therapy for CMT1X.

Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics
2025

[Analysis of clinical and novel gene mutations with X-linked Charcot-marie-tooth disease type 1].

Zhonghua yi xue za zhi
2025

Phenotype-genotype correlation in X-linked Charcot-Marie-Tooth disease: A French cohort study.

European journal of neurology
2024

Novel Missense Mutation in GJB1 Gene Leading to X-linked Charcot-Marie-Tooth Disease in Young Male: A Case Report.

Neurology India
2024

Hereditary spastic paraplegia and extensive leukoencephalopathy: a case report of a unique phenotype associated with a GJB1/Cx32 p.Pro174Ser variant.

BMC neurology
2023

X-linked Charcot Marie Tooth mutations alter CO2 sensitivity of connexin32 hemichannels.

Frontiers in cellular neuroscience
2024

Episodic Neurological Dysfunction in X-Linked Charcot-Marie-Tooth Disease: Expansion of the Phenotypic and Genetic Spectrum.

Journal of clinical neurology (Seoul, Korea)
2023

Unveiling the clinical and electrophysiological profile of CMTX6: Insights from two Brazilian families.

Journal of the peripheral nervous system : JPNS
2023

Corrigendum: Novel mutations in GJB1 trigger intracellular aggregation and stress granule formation in X-linked Charcot-Marie-Tooth Disease.

Frontiers in neuroscience
2023

Structures of wild-type and selected CMT1X mutant connexin 32 gap junction channels and hemichannels.

Science advances
2023

Gene replacement therapy in two Golgi-retained CMT1X mutants before and after the onset of demyelinating neuropathy.

Molecular therapy. Methods &amp; clinical development
2023

Pregnancy as trigger of central nervous system dysfunction in type 1 X-linked Charcot-Marie-Tooth disease.

Muscle &amp; nerve
2024

Spatial Fluctuation of Central Nervous System Lesions in X-linked Charcot-Marie-Tooth Disease with a Novel GJB1 Mutation.

Internal medicine (Tokyo, Japan)
2023

Clinical and electrophysiological characteristics of women with X-linked Charcot-Marie-Tooth disease.

European journal of neurology
2023

Mechanisms and treatment strategies of demyelinating and dysmyelinating Charcot-Marie-Tooth disease.

Neural regeneration research
2023

Charcot-Marie-Tooth Disease with a Novel Variant in Gap Junction Protein Beta 1 Presenting with Visual Field Defects.

Internal medicine (Tokyo, Japan)
2023

X-linked Charcot-Marie-Tooth disease after SARS-CoV-2 vaccination mimicked stroke-like episodes: A case report.

World journal of clinical cases
2023

A novel splicing mutation in 5'UTR of GJB1 causes X-linked Charcot-Marie-tooth disease.

Molecular genetics &amp; genomic medicine
2022

Novel mutations in GJB1 trigger intracellular aggregation and stress granule formation in X-linked Charcot-Marie-Tooth Disease.

Frontiers in neuroscience
2022

Connexin Mutations and Hereditary Diseases.

International journal of molecular sciences
2022

A Novel PRPS1 Mutation in a Japanese Patient with CMTX5.

Internal medicine (Tokyo, Japan)
2022

Structural and functional brain changes in X-linked Charcot-Marie-Tooth disease: insights from a multimodal neuroimaging study.

Neuroradiology
2021

GJB1 Gene Analysis in Two Extended Families with X-Linked Charcot-Marie-Tooth Disease.

Case reports in neurology
2021

AAV9-mediated Schwann cell-targeted gene therapy rescues a model of demyelinating neuropathy.

Gene therapy
2021

Effects of early crush on aging wild type and Connexin 32 knockout mice: Evidence for a neuroprotective state in CMT1X mouse nerve.

Journal of the peripheral nervous system : JPNS
2020

Transient, Recurrent Central Nervous System Clinical Manifestations of X-Linked Charcot-Marie-Tooth Disease Presenting with Very Long Latency Periods between Episodes: Is Prolonged Sun Exposure a Provoking Factor?

Case reports in neurological medicine
2019

GJB1 Mutation-A Disease Spectrum: Report of Case Series.

Frontiers in neurology
2020

Expansion of the phenotypic spectrum of X-linked Charcot-Marie-Tooth (CMT) disease.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
2020

Evidence for Cognitive Deficits in X-Linked Charcot-Marie-Tooth Disease.

Journal of the International Neuropsychological Society : JINS
2020

A novel GJB1 mutation associated with X-linked Charcot-Marie-Tooth disease in a large Chinese family pedigree.

Molecular genetics &amp; genomic medicine
2019

Atypical Pediatric Demyelinating Diseases of the Central Nervous System.

Current neurology and neuroscience reports
2019

A novel mutation in PRPS1 causes X-linked Charcot-Marie-Tooth disease-5.

Neuropathology : official journal of the Japanese Society of Neuropathology
2019

Gene replacement therapy after neuropathy onset provides therapeutic benefit in a model of CMT1X.

Human molecular genetics
2019

Three novel mutations in a group of Chinese patients with X-linked Charcot-Marie-Tooth disease.

Clinical neurology and neurosurgery
2019

X-linked Charcot-Marie-Tooth Disease Presenting with Stuttering Stroke-like Symptoms.

Neuropediatrics
2019

New novel mutations in Brazilian families with X-linked Charcot-Marie-Tooth disease.

Journal of the peripheral nervous system : JPNS
2019

A Novel Variant in Non-coding Region of GJB1 Is Associated With X-Linked Charcot-Marie-Tooth Disease Type 1 and Transient CNS Symptoms.

Frontiers in neurology
2019

CNS phenotype in X linked Charcot- Marie-Tooth disease.

Journal of neurology, neurosurgery, and psychiatry
2018

Acetylation of C-terminal lysines modulates protein turnover and stability of Connexin-32.

BMC cell biology
2019

X linked Charcot-Marie-Tooth disease and multiple sclerosis: emerging evidence for an association.

Journal of neurology, neurosurgery, and psychiatry
2019

X-linked Charcot-Marie-Tooth disease type 5 with recurrent weakness after febrile illness.

Brain &amp; development
2018

A novel AIFM1 mutation in a Chinese family with X-linked Charcot-Marie-Tooth disease type 4.

Neuromuscular disorders : NMD
2018

The Electrophysiological Features in X-Linked Charcot-Marie-Tooth Disease With Transient Central Nervous System Deficits.

Frontiers in neurology
2018

Genetic and phenotypic profile of 112 patients with X-linked Charcot-Marie-Tooth disease type 1.

European journal of neurology
2018

Anaesthesia and orphan diseases: anaesthetic management of a patient with X-linked Charcot-Marie-Tooth disease type 1.

European journal of anaesthesiology
2018

[Transient, recurrent, white matter lesions in X-linked Charcot-Marie-Tooth disease with heterozygote mutation of GJB1 gene: case report of a female patient].

Rinsho shinkeigaku = Clinical neurology
2018

Reversible lesions of the corpus callosum with initially restricted diffusion in a series of Caucasian children.

Pediatric radiology
2018

Coexistence of Amyotrophic Lateral Sclerosis in the Proband of an X-Linked Charcot-Marie-Tooth Disease Type 1 Pedigree in China.

Journal of clinical neurology (Seoul, Korea)
2018

Intrathecal gene therapy in mouse models expressing CMT1X mutations.

Human molecular genetics
2017

X-linked Charcot-Marie-Tooth disease with GJB1 mutation presenting as acute disseminated encephalomyelitis-like illness: A case report.

Medicine
2018

An 8-generation family with X-linked Charcot-Marie-Tooth: Confirmation Of the pathogenicity Of a 3' untranslated region mutation in GJB1 and its clinical features.

Muscle &amp; nerve
2018

Substitution impact of highly conserved arginine residue at position 75 in GJB1 gene in association with X-linked Charcot-Marie-tooth disease: A computational study.

Journal of theoretical biology
2017

Charcot-Marie-Tooth Disease 1X Simulating Paraparetic Guillain-Barre Syndrome.

The neurologist
2017

A novel missense mutation in AIFM1 results in axonal polyneuropathy and misassembly of OXPHOS complexes.

European journal of neurology
2018

Preimplantation genetic diagnosis of X-linked Charcot-Marie-Tooth disease by indirect linkage analysis.

Medicina clinica
2017

Cross-sectional analysis of a large cohort with X-linked Charcot-Marie-Tooth disease (CMTX1).

Neurology
2017

Deletion of P2 promoter of GJB1 gene a cause of Charcot-Marie-Tooth disease.

Neuromuscular disorders : NMD
2017

Clinical characterization and genetic analysis of Korean patients with X-linked Charcot-Marie-Tooth disease type 1.

Journal of the peripheral nervous system : JPNS
2017

Mutations in noncoding regions of GJB1 are a major cause of X-linked CMT.

Neurology
2017

Centrally involved X-linked Charcot-Marie-Tooth disease presenting as a stroke-mimic.

Neurology. Genetics
2016

Systemic inflammation disrupts oligodendrocyte gap junctions and induces ER stress in a model of CNS manifestations of X-linked Charcot-Marie-Tooth disease.

Acta neuropathologica communications
2016

Mutation Analysis of Gap Junction Protein Beta 1 and Genotype-Phenotype Correlation in X-linked Charcot-Marie-Tooth Disease in Chinese Patients.

Chinese medical journal
2016

CNS involvement in CMTX1 caused by a novel connexin 32 mutation: a 6-year follow-up in neuroimaging and nerve conduction.

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology
2016

Intrathecal gene therapy rescues a model of demyelinating peripheral neuropathy.

Proceedings of the National Academy of Sciences of the United States of America
2016

X-linked Charcot-Marie-Tooth disease type 6 (CMTX6) patients with a p.R158H mutation in the pyruvate dehydrogenase kinase isoenzyme 3 gene.

Journal of the peripheral nervous system : JPNS
2016

Secondary structural analysis of the carboxyl-terminal domain from different connexin isoforms.

Biopolymers
2016

A Review of X-linked Charcot-Marie-Tooth Disease.

Journal of child neurology
2016

A novel AIFM1 mutation expands the phenotype to an infantile motor neuron disease.

European journal of human genetics : EJHG
2015

Connexin: a potential novel target for protecting the central nervous system?

Neural regeneration research
2015

Intraneural GJB1 gene delivery improves nerve pathology in a model of X-linked Charcot-Marie-Tooth disease.

Annals of neurology
2015

Relapsing remitting multiple sclerosis in x-linked charcot-marie-tooth disease with central nervous system involvement.

Case reports in neurological medicine
2015

A start codon CMT1X mutation associated with transient encephalomyelitis causes complete loss of Cx32.

Neurogenetics
2015

Episodic neurological dysfunction in hereditary peripheral neuropathy.

Annals of Indian Academy of Neurology
2015

A novel mutation in GJB1 (c.212T>G) in a Chinese family with X-linked Charcot-Marie-Tooth disease.

Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia
Ver todos os 136 no EuropePMC

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Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Ordenadas pelo número de sintomas em comum.

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. Case Report: Recurrent stroke-like episodes triggered by high-altitude exposure in X-linked charcot-marie-tooth disease.
    Frontiers in genetics· 2026· PMID 41757265mais citado
  2. Patient-derived neural organoids reveal developmental impairments associated with a novel GJB1 mutation in X-linked Charcot-Marie-Tooth disease.
    Neurobiology of disease· 2026· PMID 41628834mais citado
  3. Facing the challenge of effective dosing, safety, and timing of intrathecal gene therapy for neurological disorders.
    EBioMedicine· 2026· PMID 41289961mais citado
  4. [A case of X-linked Charcot-Marie-Tooth disease type 1 (CMTX1) diagnosed based on recurrent brain lesions despite peripheral neuropathy responsive to immunotherapy].
    Rinsho shinkeigaku = Clinical neurology· 2025· PMID 41016757mais citado
  5. Random X chromosome inactivation in female Charcot-Marie-Tooth disease type X1: insights from sural nerve biopsy analysis.
    BMC neurology· 2025· PMID 40759929mais citado
  6. A Family With X-Linked Charcot-Marie-Tooth Disease Type 1: A Case for Reclassifying a Variant of Uncertain Significance in GJB1and Review of the Literature.
    J Clin Neuromuscul Dis· 2025· PMID 40901913recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:64747(Orphanet)
  2. MONDO:0018994(MONDO)
  3. GARD:12444(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q3281260(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

Charcot-Marie-Tooth ligada ao X
Compêndio · Raras BR

Charcot-Marie-Tooth ligada ao X

ORPHA:64747 · MONDO:0018994
Prevalência
1-9 / 100 000
Herança
X-linked dominant, X-linked recessive
CID-10
G60.0 · Neuropatia hereditária motora e sensorial
CID-11
Ensaios
1 ativos
Prevalência
1.6 (Europe)
MedGen
UMLS
C4551551
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

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