Hfe acts in hepatocytes to prevent hemochromatosis.
Cell Metab
; 7(2): 173-8, 2008 Feb.
Article
em En
| MEDLINE
| ID: mdl-18249176
ABSTRACT
Hereditary hemochromatosis (HH) is a prevalent, potentially fatal disorder of iron metabolism hallmarked by intestinal hyperabsorption of iron, hyperferremia, and hepatic iron overload. In both humans and mice, type I HH is associated with mutations in the broadly expressed HFE/Hfe gene. To identify where Hfe acts to prevent HH, we generated mice with tissue-specific Hfe ablations. This work demonstrates that local Hfe expression in hepatocytes serves to maintain physiological iron homeostasis, answering a long-standing question in medicine and explaining earlier clinical observations.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Antígenos de Histocompatibilidade Classe I
/
Hepatócitos
/
Hemocromatose
/
Proteínas de Membrana
Limite:
Animals
Idioma:
En
Revista:
Cell Metab
Ano de publicação:
2008
Tipo de documento:
Article