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Zebra body myopathy

ORPHA:97240DiseaseUnknownNeonatal

Ассоциированные гены (1)

ACTA1
actin alpha 1, skeletal muscle
Disease-causing germline mutation(s) in
OMIM: 102610

Фенотипы (22)

Очень частый (80–99%)21
HP:0000467Neck muscle weakness
HP:0000473Torticollis
HP:0001263Global developmental delay
HP:0001319Neonatal hypotonia
HP:0001558Decreased fetal movement
HP:0002460Distal muscle weakness
HP:0002515Waddling gait
HP:0003236Elevated circulating creatine kinase concentration
HP:0003327Axial muscle weakness
HP:0003391Gowers sign
HP:0003458EMG: myopathic abnormalities
HP:0003551Difficulty climbing stairs
HP:0003555Muscle fiber splitting
HP:0003701Proximal muscle weakness
HP:0003715Myofibrillar myopathy
HP:0003736Autophagic vacuoles
HP:0003798Nemaline bodies
HP:0003805Rimmed vacuoles
HP:0006785Limb-girdle muscular dystrophy
HP:0010628Facial palsy
HP:0012899Handgrip myotonia
Частый (30–79%)1
HP:0003713Muscle fiber necrosis

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