KEGG   DISEASE: 筋硬化症
エントリ  
H01338                                                             
名称    
筋硬化症
  上位グループ
6型コラーゲン関連筋疾患 [DS:H01341]
先天性筋ジストロフィー [DS:H00590]
概要    
Myosclerosis is an autosomal recessive disorder caused by nonsense mutation of COL6A2. This disease is one of the collagen VI myopathies, characterized by difficulty in walking in early childhood, toe walking, and progressive contractures of calf muscles. In the early 30s the muscles are slender with a firm woody consistency and associated with contractures that restrict range of motion of many joints.
カテゴリ  
神経系疾患; 筋骨格疾患
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 08 神経系の疾患
  神経筋接合部または筋の疾患
   原発性筋疾患
    8C72  先天性ミオパチー
     H01338  筋硬化症
パスウェイ 
hsa04151  PI3K-Akt signaling pathway
hsa04510  Focal adhesion
hsa04512  ECM-receptor interaction
病因遺伝子 
COL6A2 [HSA:1292] [KO:K06238]
リンク   
ICD-11: 8C72.0Y
MeSH: C564968
OMIM: 255600
文献    
  著者
Merlini L, Martoni E, Grumati P, Sabatelli P, Squarzoni S, Urciuolo A, Ferlini A, Gualandi F, Bonaldo P
  タイトル
Autosomal recessive myosclerosis myopathy is a collagen VI disorder.
  雑誌
Neurology 71:1245-53 (2008)
DOI:10.1212/01.wnl.0000327611.01687.5e
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