Alpha 1-antichymotrypsin

Protein found in humans From Wikipedia, the free encyclopedia

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.

PDBOrtholog search: PDBe RCSB
AliasesSERPINA3, AACT, ACT, GIG24, GIG25, serpin family A member 3
Quick facts SERPINA3, Available structures ...
SERPINA3
Available structures
PDBOrtholog search: PDBe RCSB
Identifiers
AliasesSERPINA3, AACT, ACT, GIG24, GIG25, serpin family A member 3
External IDsOMIM: 107280; MGI: 98377; HomoloGene: 111129; GeneCards: SERPINA3; OMA:SERPINA3 - orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_001085

NM_011458

RefSeq (protein)

NP_001076

NP_035588

Location (UCSC)Chr 14: 94.61 – 94.62 MbChr 12: 104.3 – 104.31 Mb
PubMed search[3][4]
Wikidata
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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.[6]

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.[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]

Interactions

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

See also

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

References

Further reading

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