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Hereditary sensory and autonomic neuropathy type 2

ORPHA:970DiseaseAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (4)

SCN9A
sodium voltage-gated channel alpha subunit 9
Disease-causing germline mutation(s) (loss of function) in
OMIM: 603415
WNK1
WNK lysine deficient protein kinase 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 605232
RETREG1
reticulophagy regulator 1
Disease-causing germline mutation(s) (loss of function) in
OMIM: 613114
KIF1A
kinesin family member 1A
Disease-causing germline mutation(s) (loss of function) in
OMIM: 601255

Фенотипы (15)

Очень частый (80–99%)15
HP:0000975Hyperhidrosis
HP:0001182Tapered finger
HP:0001810Dystrophic toenail
HP:0001842Acroosteolysis (feet)
HP:0002645Wormian bones
HP:0002797Osteolysis
HP:0002815Abnormality of the knee
HP:0003028Abnormality of the ankles
HP:0003103Abnormal cortical bone morphology
HP:0003202Skeletal muscle atrophy
HP:0003272Abnormality of the hip bone
HP:0003307Hyperlordosis
HP:0004349Reduced bone mineral density
HP:0005930Abnormality of epiphysis morphology
HP:0008391Dystrophic fingernails

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