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PMID:10638746

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Citation

Bennett, MJ, Lebrón, JA and Bjorkman, PJ (2000) Crystal structure of the hereditary haemochromatosis protein HFE complexed with transferrin receptor. Nature 403:46-53

Abstract

HFE is related to major histocompatibility complex (MHC) class I proteins and is mutated in the iron-overload disease hereditary haemochromatosis. HFE binds to the transferrin receptor (TfR), a receptor by which cells acquire iron-loaded transferrin. The 2.8 A crystal structure of a complex between the extracellular portions of HFE and TfR shows two HFE molecules which grasp each side of a twofold symmetric TfR dimer. On a cell membrane containing both proteins, HFE would 'lie down' parallel to the membrane, such that the HFE helices that delineate the counterpart of the MHC peptide-binding groove make extensive contacts with helices in the TfR dimerization domain. The structures of TfR alone and complexed with HFE differ in their domain arrangement and dimer interfaces, providing a mechanism for communicating binding events between TfR chains. The HFE-TfR complex suggests a binding site for transferrin on TfR and sheds light upon the function of HFE in regulating iron homeostasis.

Links

PubMed Online version:10.1038/47417

Keywords

Crystallography, X-Ray; HLA Antigens/chemistry; HLA Antigens/genetics; HLA Antigens/metabolism; Hemochromatosis/genetics; Histocompatibility Antigens Class I/chemistry; Histocompatibility Antigens Class I/genetics; Histocompatibility Antigens Class I/metabolism; Humans; Hydrogen-Ion Concentration; Iron/metabolism; Macromolecular Substances; Membrane Proteins; Models, Molecular; Mutation; Protein Binding; Protein Conformation; Receptors, Transferrin/chemistry; Receptors, Transferrin/metabolism; Transferrin/metabolism

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

HUMAN:TFR1

enables

GO:0005515: protein binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:Q30201

F

Seeded From UniProt

complete

HUMAN:HFE

located_in

GO:0005887: integral component of plasma membrane

ECO:0000304: author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

HUMAN:HFE

located_in

GO:0005886: plasma membrane

ECO:0000304: author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

HUMAN:HFE

part_of

GO:0005887: integral component of plasma membrane

ECO:0000304: author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

HUMAN:HFE

involved_in

GO:0065003: protein-containing complex assembly

ECO:0000304: author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

HUMAN:HFE

enables

GO:0005102: signaling receptor binding

ECO:0000353: physical interaction evidence used in manual assertion

UniProtKB:P02786

F

Seeded From UniProt

complete


See also

References

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