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X-linked dystonia-parkinsonism

ORPHA:53351DiseaseNot applicable, X-linked recessiveAdult

Ассоциированные гены (1)

TAF1
TATA-box binding protein associated factor 1
Disease-causing germline mutation(s) in
OMIM: 313650

Фенотипы (19)

Частый (30–79%)12
HP:0000643Blepharospasm
HP:0001304Torsion dystonia
HP:0001336Myoclonus
HP:0002067Bradykinesia
HP:0002072Chorea
HP:0002172Postural instability
HP:0002322Resting tremor
HP:0002362Shuffling gait
HP:0002378Hand tremor
HP:0002548Parkinsonism with favorable response to dopaminergic medication
HP:0004373Focal dystonia
HP:0007158Progressive extrapyramidal muscular rigidity
Периодический (5–29%)7
HP:0001288Gait disturbance
HP:0002359Frequent falls
HP:0002451Limb dystonia
HP:0006511Laryngeal stridor
HP:0010808Protruding tongue
HP:0011951Aspiration pneumonia
HP:0031162Impaired oropharyngeal swallow response

Эпидемиология (2)

Point prevalence
<1 / 1 000 000
Europe
Point prevalence
1-9 / 1 000 000
Philippines

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы