KEGG   DISEASE: 精神遅滞・眼欠損症・小顎症を伴う脳梁欠損症
エントリ  
H01035                                                             
名称    
精神遅滞・眼欠損症・小顎症を伴う脳梁欠損症
概要    
This syndrome is a condition with a unique pattern of malformations that includes coloboma (iris, optic nerve), high forehead, severe retrognathia, mental retardation, and agenesis of the corpus callosum. This syndrome is caused by mutations in 5-prime region of IGBP1 gene, a regulatory subunit of the major cellular phosphatase PP2A.
カテゴリ  
先天奇形
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  多発性の発達異常または症候群
   LD20  主な特徴として中枢神経系の異常を伴う症候群
    H01035  精神遅滞・眼欠損症・小顎症を伴う脳梁欠損症
病因遺伝子 
IGBP1 (5-prime region) [HSA:3476] [KO:K17606]
リンク   
ICD-11: LD20.Y
MeSH: C564509
OMIM: 300472
文献    
  著者
Graham JM Jr, Wheeler P, Tackels-Horne D, Lin AE, Hall BD, May M, Short KM, Schwartz CE, Cox TC
  タイトル
A new X-linked syndrome with agenesis of the corpus callosum, mental retardation, coloboma, micrognathia, and a mutation in the Alpha 4 gene at Xq13.
  雑誌
Am J Med Genet A 123A:37-44 (2003)
DOI:10.1002/ajmg.a.20504
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