KEGG   DISEASE: Phelan-McDermid 症候群
エントリ  
H01238                                                             
名称    
Phelan-McDermid 症候群
概要    
Phelan-McDermid syndrome is a genetic disorder caused by a microdeletion on chromosome 22. It is characterized by severe expressive-language delay, mild mental retardation, hypotonia, joint laxity, dolichocephaly, and minor facial dysmorphisms. It has been indicated that this syndrome may also be caused by mutations in the SHANK3/PROSAP2 gene.
カテゴリ  
染色体異常
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 20 発達異常
  遺伝子変異を除く染色体異常
   LD44  常染色体の欠失
    H01238  Phelan-McDermid 症候群
パスウェイに基づく疾患分類 [BR:jp08402]
 細胞プロセス
  nt06544  神経刺激性リガンドのシグナリング
   H01238  Phelan-McDermid 症候群
パスウェイ 
hsa04724  Glutamatergic synapse
ネットワーク
nt06544 Neuroactive ligand signaling
病因遺伝子 
SHANK3 [HSA:85358] [KO:K15009]
リンク   
ICD-11: LD44.NY
MeSH: C536801
OMIM: 606232
文献    
  著者
Bonaglia MC, Giorda R, Borgatti R, Felisari G, Gagliardi C, Selicorni A, Zuffardi O
  タイトル
Disruption of the ProSAP2 gene in a t(12;22)(q24.1;q13.3) is associated with the 22q13.3 deletion syndrome.
  雑誌
Am J Hum Genet 69:261-8 (2001)
DOI:10.1086/321293
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