ArticleProceedings of the National Academy of Sciences of the United States of America1996
beta2 knockout mice develop parenchymal iron overload: A putative role for class I genes of the major histocompatibility complex in iron metabolism.
Article in Proceedings of the National Academy of Sciences of the United States of America, 1996. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 194 citations in OpenAlex.
- TMEM119+ microglia MHC class I restricted antigen presentation impacts CD8 T cell memory, effector status, and blood-brain barrier disruption during neurotropic virus infection.Research square · 2026Article
- The oral ferroportin inhibitor vamifeport prevents liver iron overload in a mouse model of hemochromatosis.HemaSphere · 2024Article
- Hypoimmunogenic human pluripotent stem cells are valid cell sources for cell therapeutics with normal self-renewal and multilineage differentiation capacity.Stem cell research & therapy · 2023Article
- Review
- Creating class I MHC-null pigs using guide RNA and the Cas9 endonuclease.Journal of immunology (Baltimore, Md. : 1950) · 2014Article
- MHC-I expression renders catecholaminergic neurons susceptible to T-cell-mediated degeneration.Nature communications · 2014Article
- Inhibition of β2-microglobulin/hemochromatosis enhances radiation sensitivity by induction of iron overload in prostate cancer cells.PloS one · 2013Article
- Neuroimmune regulation of alcohol consumption: behavioral validation of genes obtained from genomic studies.Addiction biology · 2012Article
- β2-microglobulin induces epithelial to mesenchymal transition and confers cancer lethality and bone metastasis in human cancer cells.Cancer research · 2011Article
- Factors influencing disease phenotype and penetrance in HFE haemochromatosis.Human genetics · 2010Review
- Development of novel major histocompatibility complex class I and class II-deficient NOD-SCID IL2R gamma chain knockout mice for modeling human xenogeneic graft-versus-host disease.Methods in molecular biology (Clifton, N.J.) · 2010Article
- Beta2-microglobulin-dependent bacterial clearance and survival during murine Klebsiella pneumoniae bacteremia.Infection and immunity · 2009Article
- Function of the hemochromatosis protein HFE: Lessons from animal models.World journal of gastroenterology · 2008Article
- Iron overload and immunity.World journal of gastroenterology · 2007Review
- Liver-gut axis in the regulation of iron homeostasis.World journal of gastroenterology · 2007Review
- An impaired breeding phenotype in mice with a genetic deletion of beta-2 microglobulin and diminished MHC class I expression: role in reproductive fitness.Biology of reproduction · 2007Article
- Comparative study between Hfe-/- and beta2m-/- mice: progression with age of iron status and liver pathology.International journal of experimental pathology · 2006Article
- Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload.The American journal of pathology · 2004Article
- Mechanisms of HFE-induced regulation of iron homeostasis: Insights from the W81A HFE mutation.Proceedings of the National Academy of Sciences of the United States of America · 2003Article
- The haemochromatosis protein HFE induces an apparent iron-deficient phenotype in H1299 cells that is not corrected by co-expression of beta 2-microglobulin.The Biochemical journal · 2003Article
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Authors and funding
2 authors at 1 institution in 1 country.
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Abstract
Hemochromatosis (HC) is an inherited disorder of iron absorption, mapping within the human major histocompatibility complex (MHC). We have identified a multigene system in the murine MHC that contains excellent candidates for the murine equivalent of the human HC locus and implicate nonclassical class I genes in the control of iron absorption. This gene system is characterized by multiple copies of two head-to-head genes encoded on opposite strands and driven by one common regulatory motif. This regulatory motif has a striking homology to the promoter region of the beta-globin gene, a gene obviously involved in iron metabolism and hence termed beta-globin analogous promoter (betaGAP). Upstream of the betaGAP sequence are nonclassical class I genes. At least one of these nonclassical class I genes, Q2, is expressed in the gastrointestinal tract, the primary site of iron absorption. Also expressed in the gastrointestinal tract and downstream of the betaGAP motif is a second set of putative genes, termed Hephaestus (HEPH). Based on these observations, we hypothesized that the genes that seem to be controlled by the betaGAP regulatory motifs would be responsible for the control of Fe absorption. As a test of this hypothesis, we predicted that mice which have altered expression of class I gene products, the beta2-microglobulin knockout mice, [beta2m(-/-)], would develop Fe overload. This prediction was confirmed, and these results indicate beta2m-associated proteins are involved in the control of intestinal Fe absorption.
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