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DPM3-CDG

ORPHA:263494DiseaseAutosomal recessiveChildhood

Ассоциированные гены (1)

DPM3
dolichyl-phosphate mannosyltransferase subunit 3, regulatory
Disease-causing germline mutation(s) in
OMIM: 605951

Фенотипы (15)

Очень частый (80–99%)1
HP:0012363Decreased sialylation of O-linked protein glycosylation
Частый (30–79%)14
HP:0001315Reduced tendon reflexes
HP:0001324Muscle weakness
HP:0001644Dilated cardiomyopathy
HP:0001763Pes planus
HP:0002187Intellectual disability, profound
HP:0002401Stroke-like episode
HP:0002910Elevated circulating hepatic transaminase concentration
HP:0003487Babinski sign
HP:0003560Muscular dystrophy
HP:0003749Pelvic girdle muscle weakness
HP:0003805Rimmed vacuoles
HP:0008331Elevated creatine kinase after exercise
HP:0008981Calf muscle hypertrophy
HP:0100749Chest pain

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