KEGG   DISEASE: 外胚葉異形成症を伴わない免疫不全症
エントリ  
H01245                                                             
名称    
外胚葉異形成症を伴わない免疫不全症
概要    
A NEMO mutant causing immunodeficiency without any sign of anhidrotic ectodermal dysplasia (EDA) has been identified. The mutation, which altered the exon 9 splice site, was present in cells of ectodermal and hematopoietic origin and resulted in a heterogeneous mixture of mutant and wild-type cDNA species.
カテゴリ  
原発性免疫不全症
階層分類  
ICD-11 による疾患分類 [BR:jp08403]
 04 免疫系の疾患
  原発性免疫不全症
   4A0Y  その他の明示された原発性免疫不全症
    H01245  外胚葉異形成症を伴わない免疫不全症
パスウェイ 
hsa04659  Th17 cell differentiation
hsa04658  Th1 and Th2 cell differentiation
hsa04668  TNF signaling pathway
hsa04620  Toll-like receptor signaling pathway
病因遺伝子 
IKBKG [HSA:8517] [KO:K07210]
リンク   
ICD-11: 4A0Y
MeSH: C536289
OMIM: 300584
文献    
  著者
Courtois G, Smahi A
  タイトル
NF-kappaB-related genetic diseases.
  雑誌
Cell Death Differ 13:843-51 (2006)
DOI:10.1038/sj.cdd.4401841
文献    
  著者
Niehues T, Reichenbach J, Neubert J, Gudowius S, Puel A, Horneff G, Lainka E, Dirksen U, Schroten H, Doffinger R, Casanova JL, Wahn V
  タイトル
Nuclear factor kappaB essential modulator-deficient child with immunodeficiency yet without anhidrotic ectodermal dysplasia.
  雑誌
J Allergy Clin Immunol 114:1456-62 (2004)
DOI:10.1016/j.jaci.2004.08.047
文献    
  著者
Orange JS, Levy O, Brodeur SR, Krzewski K, Roy RM, Niemela JE, Fleisher TA, Bonilla FA, Geha RS
  タイトル
Human nuclear factor kappa B essential modulator mutation can result in immunodeficiency without ectodermal dysplasia.
  雑誌
J Allergy Clin Immunol 114:650-6 (2004)
DOI:10.1016/j.jaci.2004.06.052
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