title: Discovery of the hemochromatosis gene will require rethinking the regulation of iron metabolism.
abstract: The identity of the protein responsible for hemochromatosis, the iron overload disease, has eluded scientists for years. However, a recent report identifies the gene where the hemochromatosis defect lies. It is a gene that encodes a major histocompatibility complex (MHC) class-1-like protein called HLA-H. The mechanism by which an HLA-H defect alters iron metabolism is still unidentified. However, this new discovery will certainly ignite a new wave of study into the physiology of iron metabolism and its regulation.
authors.affiliation: Jean Mayer Human Nutrition Research Center on Aging, Tufts University, Boston MA 02111, USA.
authors.forename: J C
authors.initials: JC
authors.lastname: Fleet
mesh_terms: D000818:Animals; D002874:Chromosome Mapping; D002896:Chromosomes, Human, Pair 6; D004195:Disease Models, Animal; D008040:Genetic Linkage; D006680:HLA Antigens / Q000502:physiology; D006432:Hemochromatosis / Q000453:epidemiology / Q000235:genetics* / Q000378:metabolism; D000071020:Hemochromatosis Protein; D015395:Histocompatibility Antigens Class I / Q000502:physiology; D006801:Humans; D007501:Iron / Q000378:metabolism*; D008565:Membrane Proteins; D051379:Mice; D009154:Mutation
chemical_list: C103673:HFE protein, human; D006680:HLA Antigens; D000071020:Hemochromatosis Protein; C499535:Hfe protein, mouse; D015395:Histocompatibility Antigens Class I; D008565:Membrane Proteins; D007501:Iron