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X-linked Charcot-Marie-Tooth disease type 5

ORPHA:99014DiseaseX-linked recessiveChildhood, Infancy

Ассоциированные гены (1)

PRPS1
phosphoribosyl pyrophosphate synthetase 1
Disease-causing germline mutation(s) in
OMIM: 311850

Фенотипы (19)

Очень частый (80–99%)9
HP:0000365Hearing impairment
HP:0000648Optic atrophy
HP:0000763Sensory neuropathy
HP:0001284Areflexia
HP:0001324Muscle weakness
HP:0001761Pes cavus
HP:0003712Skeletal muscle hypertrophy
HP:0009830Peripheral neuropathy
HP:0040129Abnormal nerve conduction velocity
Частый (30–79%)1
HP:0007328Impaired pain sensation
Периодический (5–29%)9
HP:0001251Ataxia
HP:0001260Dysarthria
HP:0001262Excessive daytime somnolence
HP:0001288Gait disturbance
HP:0001337Tremor
HP:0002385Paraparesis
HP:0002463Language impairment
HP:0002650Scoliosis
HP:0002808Kyphosis

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Worldwide
Cases/families
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы