Alpha 1-antichymotrypsin

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Serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3

PDB rendering based on 1as4.
Available structures
PDB Ortholog search: PDBe, RCSB
External IDs OMIM107280 MGI105045 HomoloGene111129 ChEMBL: 5960 GeneCards: SERPINA3 Gene
RNA expression pattern
PBB GE SERPINA3 202376 at tn.png
More reference expression data
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.


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]


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


Further reading[edit]

External links[edit]