InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: A2ML1

Summary

InnateDB Protein

IDBP-17396.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

A2ML1

Protein Name

alpha-2-macroglobulin-like 1

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000299698

InnateDB Gene

IDBG-17392 (A2ML1)

Protein Structure

UniProt Annotation

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. {ECO:0000250UniProtKB:P01023, ECO:0000269PubMed:16298998}.

Subcellular Localization

Secreted {ECO:0000269PubMed:16298998}.

Disease Associations

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. {ECO:0000269PubMed:16298998}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 2 experimentally validated interaction(s) in this database.

Experimentally validated
Total	2 [view]
Protein-Protein	2 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0004866	endopeptidase inhibitor activity
GO:0004867	serine-type endopeptidase inhibitor activity
GO:0030414	peptidase inhibitor activity

Biological Process

GO:0010951	negative regulation of endopeptidase activity
GO:0052548	regulation of endopeptidase activity

Cellular Component

GO:0005576	extracellular region
GO:0005615	extracellular space
GO:0070062	extracellular vesicular exosome

Protein Structure and Domains

PDB ID

InterPro

IPR001599 Alpha-2-macroglobulin
IPR002890 Alpha-2-macroglobulin, N-terminal
IPR007990 Seminal vesicle autoantigen
IPR008930 Terpenoid cyclases/protein prenyltransferase alpha-alpha toroid
IPR009048 Alpha-macroglobulin, receptor-binding
IPR011625 Alpha-2-macroglobulin, N-terminal 2
IPR011626 Alpha-macroglobulin complement component
IPR019565 Alpha-2-macroglobulin, thiol-ester bond-forming

PFAM