KEGG   DISEASE: ReNU 症候群
エントリ  
H02804                                                             
名称    
ReNU 症候群
概要    
ReNU syndrome is a neurodevelopmental disorder characterized by intellectual disability, microcephaly, short stature, hypotonia, seizures and motor delay. It has been reported that mutations in RNU4-2 cause this syndrome. RNU4-2 is one of the genes encoding the U4 small nuclear RNA (snRNA), a critical component of the spliceosome.
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
  LD90  関連する臨床的特徴として知的発達障害を伴う病状
   H02804  ReNU 症候群
病因遺伝子 
RNU4-2 [HSA:26834] [KO:K14278]
リンク   
ICD-11: LD90.Y
OMIM: 620851
文献    
  著者
Greene D, Thys C, Berry IR, Jarvis J, Ortibus E, Mumford AD, Freson K, Turro E
  タイトル
Mutations in the U4 snRNA gene RNU4-2 cause one of the most prevalent monogenic neurodevelopmental disorders.
  雑誌
Nat Med 10.1038/s41591-024-03085-5 (2024)
DOI:10.1038/s41591-024-03085-5
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