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Adult-onset multiple mitochondrial DNA deletion syndrome due to DGUOK deficiency

ORPHA:329314DiseaseAutosomal recessiveAdult

Ассоциированные гены (1)

DGUOK
deoxyguanosine kinase
Disease-causing germline mutation(s) in
OMIM: 601465

Фенотипы (21)

Частый (30–79%)8
HP:0000590Progressive external ophthalmoplegia
HP:0001488Bilateral ptosis
HP:0002015Dysphagia
HP:0003325Limb-girdle muscle weakness
HP:0003390Sensory axonal neuropathy
HP:0003797Limb-girdle muscle atrophy
HP:0007340Lower limb muscle weakness
HP:0008615Adult onset sensorineural hearing impairment
Периодический (5–29%)13
HP:0000486Strabismus
HP:0000518Cataract
HP:0000648Optic atrophy
HP:0000716Depression
HP:0000726Dementia
HP:0001251Ataxia
HP:0001618Dysphonia
HP:0003326Myalgia
HP:0003394Muscle spasm
HP:0003558Viral infection-induced rhabdomyolysis
HP:0003749Pelvic girdle muscle weakness
HP:0025406Asthenia
HP:0100543Cognitive impairment

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы