KEGG   DISEASE: Birk-Landau-Perez 症候群
エントリ  
H02550                                                             
名称    
Birk-Landau-Perez 症候群
概要    
Birk-Landau-Perez syndrome (BILAPES) is a novel autosomal recessive cerebro-renal syndrome characterized by early onset intellectual disability and tubulointerstitial nephropathy. It has been suggested that SLC30A9 mutation affecting intracellular zinc homeostasis causes this disease. SLC30A9 encodes a putative zinc transporter.
カテゴリ  
先天性代謝異常症
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 05 内分泌, 栄養, 代謝の疾患
  代謝疾患
   代謝物質の吸収または輸送の疾患
    5C64  ミネラルの吸収または輸送の疾患
     H02550  Birk-Landau-Perez 症候群
病因遺伝子 
SLC30A9 [HSA:10463] [KO:K14696]
リンク   
ICD-11: 5C64.2Y
OMIM: 617595
文献    
  著者
Perez Y, Shorer Z, Liani-Leibson K, Chabosseau P, Kadir R, Volodarsky M, Halperin D, Barber-Zucker S, Shalev H, Schreiber R, Gradstein L, Gurevich E, Zarivach R, Rutter GA, Landau D, Birk OS
  タイトル
SLC30A9 mutation affecting intracellular zinc homeostasis causes a novel cerebro-renal syndrome.
  雑誌
Brain 140:928-939 (2017)
DOI:10.1093/brain/awx013
LinkDB    

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