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Autosomal recessive spastic paraplegia type 48

ORPHA:306511DiseaseAutosomal recessiveAdult

Ассоциированные гены (1)

AP5Z1
adaptor related protein complex 5 subunit zeta 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613653

Фенотипы (19)

Очень частый (80–99%)2
HP:0002079Hypoplasia of the corpus callosum
HP:0007020Progressive spastic paraplegia
Частый (30–79%)13
HP:0000020Urinary incontinence
HP:0001249Intellectual disability
HP:0001251Ataxia
HP:0001336Myoclonus
HP:0001347Hyperreflexia
HP:0002061Lower limb spasticity
HP:0002064Spastic gait
HP:0002839Urinary bladder sphincter dysfunction
HP:0003236Elevated circulating creatine kinase concentration
HP:0003319Abnormality of the cervical spine
HP:0007340Lower limb muscle weakness
HP:0030890Hyperintensity of cerebral white matter on MRI
HP:0100543Cognitive impairment
Периодический (5–29%)4
HP:0000488Retinopathy
HP:0001300Parkinsonism
HP:0002136Broad-based gait
HP:0009830Peripheral neuropathy

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