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Distal arthrogryposis type 1

ORPHA:1146Malformation syndromeAutosomal dominantAntenatal, Neonatal

Ассоциированные гены (6)

NALCN
sodium leak channel, non-selective
Disease-causing germline mutation(s) in
OMIM: 611549
TNNI2
troponin I2, fast skeletal type
Disease-causing germline mutation(s) in
OMIM: 191043
TNNT3
troponin T3, fast skeletal type
Disease-causing germline mutation(s) in
OMIM: 600692
TPM2
tropomyosin 2
Disease-causing germline mutation(s) in
OMIM: 190990
MYH3
myosin heavy chain 3
Disease-causing germline mutation(s) in
OMIM: 160720
MYBPC1
myosin binding protein C1
Disease-causing germline mutation(s) in
OMIM: 160794

Фенотипы (9)

Очень частый (80–99%)2
HP:0001181Adducted thumb
HP:0010557Overlapping fingers
Частый (30–79%)4
HP:0001387Joint stiffness
HP:0001883Talipes
HP:0009465Ulnar deviation of finger
HP:0100490Camptodactyly of finger
Периодический (5–29%)3
HP:0000160Narrow mouth
HP:0001838Rocker bottom foot
HP:0003272Abnormality of the hip bone

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы