AMBP

Description

The AMBP (alpha-1-microglobulin/bikunin precursor) is a protein-coding gene located on chromosome 9.

Protein AMBP is a protein that in humans is encoded by the AMBP gene.

Alpha-1-microglobulin is an antioxidant and tissue repair protein with reductase, heme-binding, and radical-scavenging activities. It removes and protects against harmful oxidants and repairs macromolecules in intravascular and extravascular spaces and in intracellular compartments. Intravascularly, it plays a regulatory role in red cell homeostasis by preventing heme- and reactive oxygen species-induced cell damage. It binds and degrades free heme to protect fetal and adult red blood cells from hemolysis. It reduces extracellular methemoglobin, a Fe3+ (ferric) form of hemoglobin that cannot bind oxygen, back to the Fe2+ (ferrous) form deoxyhemoglobin, which has oxygen-carrying potential. Upon acute inflammation, it inhibits oxidation of low-density lipoprotein particles by MPO and limits vascular damage. Extravascularly, it protects from oxidation products formed on extracellular matrix structures and cell membranes. It catalyzes the reduction of carbonyl groups on oxidized collagen fibers and preserves cellular and extracellular matrix ultrastructures. Importantly, it counteracts the oxidative damage at blood-placenta interface, preventing leakage of free fetal hemoglobin into the maternal circulation. Intracellularly, it has a role in maintaining mitochondrial redox homeostasis. Bound to complex I of the respiratory chain of mitochondria, it may scavenge free radicals and preserve mitochondrial ATP synthesis. It protects renal tubule epithelial cells from heme-induced oxidative damage to mitochondria. It reduces cytochrome c from Fe3+ (ferric) to the Fe2+ (ferrous) state through formation of superoxide anion radicals in the presence of ascorbate or NADH/NADPH electron donor cofactors, ascorbate being the preferred cofactor. It has a chaperone role in facilitating the correct folding of bikunin in the endoplasmic reticulum compartment. {ECO:0000250|UniProtKB:Q07456, ECO:0000269|PubMed:11877257, ECO:0000269|PubMed:15683711, ECO:0000269|PubMed:21356557, ECO:0000269|PubMed:22096585, ECO:0000269|PubMed:23157686, ECO:0000269|PubMed:23642167, ECO:0000269|PubMed:25698971, ECO:0000269|PubMed:32092412, ECO:0000269|PubMed:32823731}

AMBP is also known as A1M, EDC1, HCP, HI30, IATIL, ITI, ITIL, ITILC, UTI.

Associated Diseases

WES Whole Exome Sequencing