Hemochromatosis type 2B-HFE2 (JHV) locus: 1q21 [§§] is caused by mutation in the (HAMP) hepcidin gene, hemojuvelin disrupt transferrin-bound irons ability to stimulate expression and may influence the phenotype with adult-onset HFE hemochromatosis in the state of JH (heavy-chain-diversity joining region (JH) immunoglobulin gene) even at a young age, mainly due to chromosome 1q-linked juvenile hemochromatosis. Hemojuvelin acts through the multifunctional (BMP) bone morphogenetic protein pathway and neogenin that regulates hepcidin expression and bind simultaneously. Hemojuvelin is a member of the repulsive guidance molecule (RGM) family controlled by the liver-derived peptide hepcidin mediated by the transporter DMT1 (SLC11A1) reduced by the ferric CYBRD1 cytochrome b reductase Dcytb, which display very low expression of liver hepcidin essential to maintain body iron homeostasis. Iron that is not specifically chaperoned through its essential functional pathways is damaging to biological systems.
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