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Autosomal recessive spastic paraplegia type 5A

ORPHA:100986DiseaseAutosomal recessiveAdolescent, Childhood

Ассоциированные гены (1)

CYP7B1
cytochrome P450 family 7 subfamily B member 1
Disease-causing germline mutation(s) in
OMIM: 603711

Фенотипы (24)

Очень частый (80–99%)5
HP:0001258Spastic paraplegia
HP:0002061Lower limb spasticity
HP:0002495Impaired vibratory sensation
HP:0003487Babinski sign
HP:0007340Lower limb muscle weakness
Частый (30–79%)6
HP:0000079Abnormality of the urinary system
HP:0001317Abnormal cerebellum morphology
HP:0001761Pes cavus
HP:0002500Abnormal cerebral white matter morphology
HP:0007210Lower limb amyotrophy
HP:0011448Ankle clonus
Периодический (5–29%)3
HP:0002070Limb ataxia
HP:0002078Truncal ataxia
HP:0006827Atrophy of the spinal cord
Очень редкий (1–4%)10
HP:0000407Sensorineural hearing impairment
HP:0000518Cataract
HP:0000639Nystagmus
HP:0001260Dysarthria
HP:0001271Polyneuropathy
HP:0002015Dysphagia
HP:0002650Scoliosis
HP:0003484Upper limb muscle weakness
HP:0006986Upper limb spasticity
HP:0009129Upper limb amyotrophy

Эпидемиология (1)

Point prevalence
<1 / 1 000 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы