Alpha 1-antichymotrypsin

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PBB Protein SERPINA3 image.jpg
Available structures
PDBOrtholog search: PDBe RCSB
AliasesSERPINA3, AACT, ACT, GIG24, GIG25, serpin family A member 3
External IDsMGI: 98377 HomoloGene: 111129 GeneCards: SERPINA3
Gene location (Human)
Chromosome 14 (human)
Chr.Chromosome 14 (human)[1]
Chromosome 14 (human)
Genomic location for SERPINA3
Genomic location for SERPINA3
Band14q32.13Start94,612,377 bp[1]
End94,624,055 bp[1]
RNA expression pattern
PBB GE SERPINA3 202376 at fs.png
More reference expression data
RefSeq (mRNA)



RefSeq (protein)



Location (UCSC)Chr 14: 94.61 – 94.62 MbChr 12: 104.34 – 104.35 Mb
PubMed search[3][4]
View/Edit HumanView/Edit Mouse

Alpha 1-antichymotrypsin (symbol α1AC,[5] A1AC, or a1ACT) 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.[6]

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.[7]

Alpha 1-antichymotrypsin is also associated with the pathogenesis of Alzheimer's disease as it enhances the formation of amyloid-fibrils in this disease.[6]


Alpha 1-antichymotrypsin has been shown to interact with DNAJC1.[8]

See also[edit]

  • Alpha-1 antitrypsin, another serpin that is analogous for protecting the body from excessive effects of its own inflammatory proteases


  1. ^ a b c GRCh38: Ensembl release 89: ENSG00000196136 - Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000058207 - Ensembl, May 2017
  3. ^ "Human PubMed Reference:".
  4. ^ "Mouse PubMed Reference:".
  5. ^ Logan, Carolynn M.; Rice, M. Katherine (1987). Logan's Medical and Scientific Abbreviations. Philadelphia: J. B. Lippincott Company. p. 3. ISBN 0-397-54589-4.
  6. ^ a b Kalsheker N (1996). "Alpha 1-antichymotrypsin". Int. J. Biochem. Cell Biol. 28 (9): 961–4. doi:10.1016/1357-2725(96)00032-5. PMID 8930118.
  7. ^ "Entrez Gene: SERPINA3 serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3".
  8. ^ Kroczynska B, Evangelista CM, Samant SS, Elguindi EC, Blond SY (March 2004). "The SANT2 domain of the murine tumor cell DnaJ-like protein 1 human homologue interacts with alpha1-antichymotrypsin and kinetically interferes with its serpin inhibitory activity". J. Biol. Chem. 279 (12): 11432–43. doi:10.1074/jbc.M310903200. PMC 1553221. PMID 14668352.

Further reading[edit]

External links[edit]