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alpha-1-antichymotrypsin; ACT; cell growth-inhibiting gene 24/25 protein; Serpin A3 (SERPINA3, AACT, GIG24, GIG25)

Function: - physiological function is unclear - inhibits neutrophil cathepsin G & mast cell chymase, both of which can convert angiotensin-1 to the active angiotensin-2 - interacts with DNAJC1 Structure: - the reactive center loop (RCL) extends out from the body of the protein & directs binding to the target protease - the protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site & the Ser hydroxyl of the protease - resulting inactive serpin-protease complex is highly stable - belongs to the serpin family Compartment: - secreted - occurs normally in bronchial secretions Expression: - plasma - synthesized in the liver - concentration increases in the acute phase of inflammation or infection. found in the amyloid Pathology: - found in senile plaques of patients with Alzheimer's disease, thus may be secreted by neurons, glial cells, or the choroid epithelium Notes: - alpha-1-antichymotrypsin can bind DNA - uncertain whether Met-1 or Met-4 is the initiator

Interactions

molecular events

Related

alpha-1 antichymotrypsin Ag in tissue alpha-1 antichymotrypsin in specimen

General

acute phase protein glycoprotein inter alpha globulin serine protease inhibitor; serpin

Properties

SIZE: entity length = 423 aa MW = 48 kD COMPARTMENT: plasma MOTIF: signal sequence {1-23} N-glycosylation site {N33} N-glycosylation site {N93} N-glycosylation site {N106} N-glycosylation site {N127} N-glycosylation site {N186} DNA-binding motif SITE: 235-237 N-glycosylation site {N271} RCL {369-394} MOTIF: peptide motif {383-384} INHIBITS: serine protease SECRETED-BY: hepatocyte epithelial cell WITHIN: bronchus

Database Correlations

OMIM 107280 UniProt P01011 Pfam PF00079 Entrez Gene 12 Kegg hsa:12; -.

References

  1. Entrez Gene :accession 12
  2. UniProt :accession P01011
  3. Wikipedia; Note: alpha-1 antichymotrypsin entry http://en.wikipedia.org/wiki/alpha_1-antichymotrypsin

Component-of

molecular complex