From Wikipedia, the free encyclopedia
Protein-coding gene in the species Homo sapiens
Alpha 1-antichymotrypsin (symbol α1 AC ,
[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 .
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
^
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 . Philadelphia:
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 .
Further reading
External links
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