Alpha 1-antichymotrypsin

From Wikipedia, the free encyclopedia
Jump to: navigation, search
Serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3

PDB rendering based on 1as4.
Available structures
PDB Ortholog search: PDBe, RCSB
Identifiers
Symbols SERPINA3 ; AACT; ACT; GIG25
External IDs OMIM107280 MGI105045 HomoloGene111129 ChEMBL: 5960 GeneCards: SERPINA3 Gene
RNA expression pattern
PBB GE SERPINA3 202376 at tn.png
More reference expression data
Orthologs
Species Human Mouse
Entrez 12 20716
Ensembl ENSG00000196136 ENSMUSG00000021091
UniProt P01011 G3X8T9
RefSeq (mRNA) NM_001085 NM_009252
RefSeq (protein) NP_001076 NP_033278
Location (UCSC) Chr 14:
95.06 – 95.09 Mb
Chr 12:
104.41 – 104.41 Mb
PubMed search [1] [2]

Alpha 1-antichymotrypsin is an alpha globulin glycoprotein that is a member of the serpin superfamily. In humans, it is encoded by the SERPINA3 gene.

Function[edit]

Alpha 1-antichymotrypsin inhibits the activity of certain enzymes called proteases, such as cathepsin G that is found in neutrophils, and chymases found in mast cells, by cleaving them into a different shape or conformation. This activity protects some tissues, such as the lower respiratory tract, from damage caused by proteolytic enzymes.[1]

This protein is produced in the liver, and is an acute phase protein that is induced during inflammation.

Clinical significance[edit]

Deficiency of this protein has been associated with liver disease. Mutations have been identified in patients with Parkinson disease and chronic obstructive pulmonary disease.[2]

Alpha 1-antichymotrypsin is also associated with the pathogenesis of Alzheimer's disease as it enhances the formation of amyloid-fibrils in this disease.[1]

Interactions[edit]

Alpha 1-antichymotrypsin has been shown to interact with DNAJC1.[3]

References[edit]

Further reading[edit]

External links[edit]