A8K2U0.3
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
105,968 Da
NCBI Official Full Name
Alpha-2-macroglobulin-like protein 1
NCBI Official Synonym Full Names
alpha-2-macroglobulin like 1
NCBI Official Synonym Symbols
NCBI Protein Information
alpha-2-macroglobulin-like protein 1
UniProt Protein Name
Alpha-2-macroglobulin-like protein 1
UniProt Synonym Protein Names
C3 and PZP-like alpha-2-macroglobulin domain-containing protein 9
UniProt Entry Name
A2ML1_HUMAN
NCBI Summary for A2ML1
This gene encodes a member of the alpha-macroglobulin superfamily. The encoded protein is thought to be an N-glycosylated monomeric protein that acts as an inhibitor of several proteases. It has been shown to form covalent interactions with proteases, and has been reported as the p170 antigen recognized by autoantibodies in the autoimmune disease paraneoplastic pemphigus (PNP; PMID:20805888). Mutations in these gene have also been associated with some cases of Noonan syndrome (NS; PMID:24939586) as well as some cases of otitis media (PMID:26121085). Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Aug 2015]
UniProt Comments for A2ML1
A2ML1: Is able to inhibit all four classes of proteinases by a unique 'trapping' mechanism. This protein has a peptide stretch, called the 'bait region' which contains specific cleavage sites for different proteinases. When a proteinase cleaves the bait region, a conformational change is induced in the protein which traps the proteinase. The entrapped enzyme remains active against low molecular weight substrates (activity against high molecular weight substrates is greatly reduced). Following cleavage in the bait region a thioester bond is hydrolyzed and mediates the covalent binding of the protein to the proteinase. Displays inhibitory activity against chymotrypsin, papain, thermolysin, subtilisin A and, to a lesser extent, elastase but not trypsin. May play an important role during desquamation by inhibiting extracellular proteases. Belongs to the protease inhibitor I39 (alpha-2- macroglobulin) family.
Protein type: Secreted, signal peptide; Secreted; Inhibitor
Chromosomal Location of Human Ortholog: 12p13.31
Cellular Component: extracellular space
Molecular Function: protease inhibitor activity
Biological Process: regulation of endopeptidase activity
Research Articles on A2ML1
Precautions
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Diseases associated with A2ML1 blocking peptide
Organs/Tissues associated with A2ML1 blocking peptide
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