← Назад

NARP syndrome

ORPHA:644DiseaseMitochondrial inheritanceChildhood

Ассоциированные гены (1)

MT-ATP6
mitochondrially encoded ATP synthase membrane subunit 6
Disease-causing germline mutation(s) in
OMIM: 516060

Фенотипы (28)

Частый (30–79%)27
HP:0000365Hearing impairment
HP:0000510Rod-cone dystrophy
HP:0000543Optic disc pallor
HP:0000618Blindness
HP:0000639Nystagmus
HP:0000726Dementia
HP:0000737Irritability
HP:0000763Sensory neuropathy
HP:0001133Constriction of peripheral visual field
HP:0001136Retinal arteriolar tortuosity
HP:0001250Seizure
HP:0001251Ataxia
HP:0001263Global developmental delay
HP:0002119Ventriculomegaly
HP:0002120Cerebral cortical atrophy
HP:0002315Headache
HP:0003394Muscle spasm
HP:0003487Babinski sign
HP:0003701Proximal muscle weakness
HP:0003739Myoclonic spasms
HP:0004322Short stature
HP:0007117Corticospinal tract atrophy
HP:0007240Progressive gait ataxia
HP:0007814Retinal pigment epithelial mottling
HP:0010864Intellectual disability, severe
HP:0012751Abnormal basal ganglia MRI signal intensity
HP:0030588Abnormal visual field test
Исключён (0%)1
HP:0008316Abnormal mitochondria in muscle tissue

Эпидемиология (1)

Point prevalence
1-9 / 100 000
Europe

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы