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DNA2-related mitochondrial DNA deletion syndrome

ORPHA:352470DiseaseAutosomal dominantAll ages

Ассоциированные гены (1)

DNA2
DNA replication helicase/nuclease 2
Disease-causing germline mutation(s) in
OMIM: 601810

Фенотипы (20)

Очень частый (80–99%)2
HP:0000590Progressive external ophthalmoplegia
HP:0003325Limb-girdle muscle weakness
Частый (30–79%)18
HP:0003198Myopathy
HP:0003307Hyperlordosis
HP:0003326Myalgia
HP:0003391Gowers sign
HP:0003394Muscle spasm
HP:0003737Mitochondrial myopathy
HP:0004673Decreased facial expression
HP:0007970Congenital ptosis
HP:0008331Elevated creatine kinase after exercise
HP:0040013Decreased mitochondrial number
HP:0000716Depression
HP:0001288Gait disturbance
HP:0001290Generalized hypotonia
HP:0001533Slender build
HP:0001558Decreased fetal movement
HP:0002828Multiple joint contractures
HP:0002870Obstructive sleep apnea
HP:0002875Exertional dyspnea

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