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Autosomal dominant Charcot-Marie-Tooth disease type 2W

ORPHA:488333DiseaseAutosomal dominantAdolescent, Adult, Childhood

Ассоциированные гены (1)

HARS1
histidyl-tRNA synthetase 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 142810

Фенотипы (20)

Очень частый (80–99%)5
HP:0002166Impaired vibration sensation in the lower limbs
HP:0003474Somatic sensory dysfunction
HP:0007108Demyelinating peripheral neuropathy
HP:0008959Distal upper limb muscle weakness
HP:0009053Distal lower limb muscle weakness
Частый (30–79%)6
HP:0001288Gait disturbance
HP:0001348Brisk reflexes
HP:0001760Abnormal foot morphology
HP:0001761Pes cavus
HP:0003376Steppage gait
HP:0007002Motor axonal neuropathy
Периодический (5–29%)9
HP:0001765Hammertoe
HP:0003100Slender long bone
HP:0003438Absent Achilles reflex
HP:0006937Impaired distal tactile sensation
HP:0007328Impaired pain sensation
HP:0008954Intrinsic hand muscle atrophy
HP:0012531Pain
HP:0030051Tip-toe gait
HP:0030237Hand muscle weakness

Эпидемиология (2)

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

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы