Protein-coding gene in the species Homo sapiens
Alpha 1-antichymotrypsin (symbolα1 AC ,[ 5] A1AC , ora1ACT ) is analpha globulin glycoprotein that is a member of theserpin superfamily. In humans, it is encoded by theSERPINA3 gene .
Alpha 1-antichymotrypsin inhibits the activity of certainenzymes calledproteases , such ascathepsin G that is found inneutrophils , andchymases found inmast cells , by cleaving them into a different shape orconformation . This activity protects some tissues, such as thelower respiratory tract , from damage caused byproteolytic enzymes.[ 6]
This protein is produced in theliver , and is anacute phase protein that is induced duringinflammation .
Clinical significance [ edit ] Deficiency of this protein has been associated withliver disease . Mutations have been identified in patients withParkinson disease andchronic obstructive pulmonary disease .[ 7]
Alpha 1-antichymotrypsin is also associated with thepathogenesis ofAlzheimer's disease as it enhances the formation of amyloid-fibrils in this disease.[ 6]
Alpha 1-antichymotrypsin has been shown tointeract withDNAJC1 .[ 8]
Alpha-1 antitrypsin , another serpin that is analogous for protecting the body from excessive effects of its own inflammatory proteases^a b c GRCh38: Ensembl release 89: ENSG00000196136 –Ensembl , May 2017^a b c GRCm38: Ensembl release 89: ENSMUSG00000058207 –Ensembl , May 2017^ "Human PubMed Reference:" .National Center for Biotechnology Information, U.S. National Library of Medicine .^ "Mouse PubMed Reference:" .National Center for Biotechnology Information, U.S. National Library of Medicine .^ Logan, Carolynn M.; Rice, M. Katherine (1987).Logan's Medical and Scientific Abbreviations J. B. Lippincott Company . p. 3 .ISBN 0-397-54589-4 ^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 .S2CID 11230631 . ^ "Entrez Gene: SERPINA3 serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3" .^ 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 .
PDB gallery
1as4 : CLEAVED ANTICHYMOTRYPSIN A349R
1qmn : ALPHA1-ANTICHYMOTRYPSIN SERPIN IN THE DELTA CONFORMATION (PARTIAL LOOP INSERTION)
2ach : CRYSTAL STRUCTURE OF CLEAVED HUMAN ALPHA1-ANTICHYMOTRYPSIN AT 2.7 ANGSTROMS RESOLUTION AND ITS COMPARISON WITH OTHER SERPINS
3caa : CLEAVED ANTICHYMOTRYPSIN A347R
4caa : CLEAVED ANTICHYMOTRYPSIN T345R
