KEGG   DISEASE: 葉酸反応性巨赤芽球性貧血症
エントリ  
H02699                                                             
名称    
葉酸反応性巨赤芽球性貧血症
概要    
Folate-responsive megaloblastic anemia (MEGAF) is a severe recurrent megaloblastic anemia caused by mutations in SLC19A1. It has been reported that the laboratory abnormalities and clinical signs were normalized only after adding folic acid to the patient's therapy. SLC19A1 encodes the reduced folate carrier (RFC) that facilitates the cellular uptake of anionic folates.
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
    5C63  ビタミンまたは非タンパク質補因子の吸収または輸送の疾患
     H02699  葉酸反応性巨赤芽球性貧血症
パスウェイ 
hsa04977  Vitamin digestion and absorption
病因遺伝子 
SLC19A1 [HSA:6573] [KO:K14609]
リンク   
ICD-11: 5C63.Y
OMIM: 601775
文献    
  著者
Svaton M, Skvarova Kramarzova K, Kanderova V, Mancikova A, Smisek P, Jesina P, Krijt J, Stiburkova B, Dobrovolny R, Sokolova J, Bakardjieva-Mihaylova V, Vodickova E, Rackova M, Stuchly J, Kalina T, Stary J, Trka J, Fronkova E, Kozich V
  タイトル
A homozygous deletion in the SLC19A1 gene as a cause of folate-dependent recurrent megaloblastic anemia.
  雑誌
Blood 135:2427-2431 (2020)
DOI:10.1182/blood.2019003178
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