Alpha 1-antichymotrypsin

SERPINA3
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
Aliases SERPINA3, AACT, ACT, GIG24, GIG25, serpin family A member 3
External IDs MGI: 98377 HomoloGene: 111129 GeneCards: SERPINA3
RNA expression pattern
More reference expression data
Orthologs
Species Human Mouse
Entrez

12

20714

Ensembl

ENSG00000273259

ENSMUSG00000058207

UniProt

P01011

P07759

RefSeq (mRNA)

NM_001085

NM_011458

RefSeq (protein)

NP_001076.2

NP_035588.2

Location (UCSC) Chr 14: 94.59 – 94.62 Mb Chr 12: 104.34 – 104.35 Mb
PubMed search [1] [2]
Wikidata
View/Edit HumanView/Edit Mouse

Alpha 1-antichymotrypsin (α1AC)[3] is an alpha globulin glycoprotein that is a member of the serpin superfamily. In humans, it is encoded by the SERPINA3 gene.

Function

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.[4]

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

Clinical significance

Deficiency of this protein has been associated with liver disease. Mutations have been identified in patients with Parkinson disease and chronic obstructive pulmonary disease.[5]

Alpha 1-antichymotrypsin is also associated with the pathogenesis of Alzheimer's disease as it enhances the formation of amyloid-fibrils in this disease.[4]

Interactions

Alpha 1-antichymotrypsin has been shown to interact with DNAJC1.[6]

References

  1. ↑ "Human PubMed Reference:".
  2. ↑ "Mouse PubMed Reference:".
  3. ↑ 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.
  4. 1 2 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.
  5. ↑ "Entrez Gene: SERPINA3 serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3".
  6. ↑ 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 1553221Freely accessible. PMID 14668352.

Further reading

External links


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