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Charcot-Marie-Tooth disease type 1B

ORPHA:101082DiseaseAutosomal dominantAdolescent, Adult, Childhood, Infancy

Ассоциированные гены (1)

MPZ
myelin protein zero
Disease-causing germline mutation(s) in
OMIM: 159440

Фенотипы (14)

Очень частый (80–99%)1
HP:0001324Muscle weakness
Частый (30–79%)11
HP:0000365Hearing impairment
HP:0000615Abnormal pupil morphology
HP:0000762Decreased nerve conduction velocity
HP:0001284Areflexia
HP:0002650Scoliosis
HP:0002922Increased CSF protein concentration
HP:0003202Skeletal muscle atrophy
HP:0003236Elevated circulating creatine kinase concentration
HP:0003469Peripheral dysmyelination
HP:0003477Peripheral axonal neuropathy
HP:0003712Skeletal muscle hypertrophy
Периодический (5–29%)2
HP:0001270Motor delay
HP:0003474Somatic sensory dysfunction

Эпидемиология (1)

Point prevalence
1-9 / 100 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы