Crystal structure of the hemochromatosis protein HFE and characterization of its interaction with transferrin receptor.
Cell
; 93(1): 111-23, 1998 Apr 03.
Article
en En
| MEDLINE
| ID: mdl-9546397
HFE is an MHC-related protein that is mutated in the iron-overload disease hereditary hemochromatosis. HFE binds to transferrin receptor (TfR) and reduces its affinity for iron-loaded transferrin, implicating HFE in iron metabolism. The 2.6 A crystal structure of HFE reveals the locations of hemochromatosis mutations and a patch of histidines that could be involved in pH-dependent interactions. We also demonstrate that soluble TfR and HFE bind tightly at the basic pH of the cell surface, but not at the acidic pH of intracellular vesicles. TfR:HFE stoichiometry (2:1) differs from TfR:transferrin stoichiometry (2:2), implying a different mode of binding for HFE and transferrin to TfR, consistent with our demonstration that HFE, transferrin, and TfR form a ternary complex.
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Colección:
01-internacional
Base de datos:
MEDLINE
Asunto principal:
Receptores de Transferrina
/
Antígenos de Histocompatibilidad Clase I
/
Estructura Secundaria de Proteína
/
Antígenos HLA
/
Proteínas de la Membrana
Límite:
Humans
Idioma:
En
Revista:
Cell
Año:
1998
Tipo del documento:
Article
País de afiliación:
Estados Unidos
Pais de publicación:
Estados Unidos