A2ML1 Antibody – Rabbit Polyclonal

This gene encodes a member of the alpha-macroglobulin superfamily. The encoded protein acts as an inhibitor for several proteases, and has been reported as the p170 antigen recognized by autoantibodies in the autoimmune disease paraneoplastic pemphigus (PNP; PMID: 20805888). Alternative splicing results in multiple transcript variants. [provided by RefSeq, Feb 2012]

Function: 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

By similarity. 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. Ref.5

Subunit structure: Monomer. Ref.5

Subcellular location: Secreted Ref.5.

Tissue specificity: In the epidermis, expressed predominantly in the granular layer at the apical edge of keratinocytes (at protein level). Also detected in placenta, testis and thymus but not in epithelia of kidney, lung, small intestine or colon. Ref.5

Developmental stage: Up-regulated during keratinocyte differentiation. Ref.5

Sequence similarities: Belongs to the protease inhibitor I39 (alpha-2-macroglobulin) family. [View classification]

Sequence caution: The sequence BAB71612.1 differs from that shown. Reason: Erroneous initiation. The sequence BAC04793.1 differs from that shown. Reason: Erroneous initiation. The sequence BAC85653.1 differs from that shown. Reason: Erroneous initiation. The sequence BAC85654.1 differs from that shown. Reason: Erroneous initiation.