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Mitochondrial myopathy and sideroblastic anemia

ORPHA:2598DiseaseAutosomal recessiveAdolescent, Childhood, Infancy

Ассоциированные гены (2)

PUS1
pseudouridine synthase 1
Disease-causing germline mutation(s) in
OMIM: 608109
YARS2
tyrosyl-tRNA synthetase 2
Disease-causing germline mutation(s) in
OMIM: 610957

Фенотипы (19)

Очень частый (80–99%)12
HP:0000218High palate
HP:0000343Long philtrum
HP:0000347Micrognathia
HP:0001252Hypotonia
HP:0001903Anemia
HP:0001939Abnormality of metabolism/homeostasis
HP:0003128Lactic acidosis
HP:0003198Myopathy
HP:0003457EMG abnormality
HP:0003737Mitochondrial myopathy
HP:0009055Generalized limb muscle atrophy
HP:0009743Distichiasis
Частый (30–79%)7
HP:0000252Microcephaly
HP:0000501Glaucoma
HP:0000823Delayed puberty
HP:0001249Intellectual disability
HP:0002650Scoliosis
HP:0002808Kyphosis
HP:0003196Short nose

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