The transferrin receptor binding site on HFE, the class I MHC-related protein mutated in hereditary hemochromatosis.
J Mol Biol
; 289(4): 1109-18, 1999 Jun 18.
Article
em En
| MEDLINE
| ID: mdl-10369785
HFE is a class I major histocompatibility complex (MHC)-related protein that is mutated in patients with the iron storage disease hereditary hemochromatosis. HFE binds tightly to transferrin receptor (TfR), the receptor that mediates uptake of iron-loaded transferrin. The binding affinities for TfR of HFE mutants, designed using the HFE crystal structure, were measured using biosensor assays. The results allow localization of the TfR binding site on HFE to the C-terminal portion of the alpha1 domain helix and an adjacent loop, a region distinct from the ligand binding sites on class I MHC and related proteins. A biosensor-derived pH-dependent affinity profile for the HFE-TfR interaction is discussed in terms of HFE's hypothesized role in intracellular trafficking.
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Base de dados:
MEDLINE
Assunto principal:
Receptores da Transferrina
/
Antígenos de Histocompatibilidade Classe I
/
Hemocromatose
/
Antígenos HLA
/
Proteínas de Membrana
Limite:
Animals
/
Humans
Idioma:
En
Ano de publicação:
1999
Tipo de documento:
Article