Alfa 1-antichymotrypsin

Alfa 1-antichymotrypsin
SERPINA3
SI-enheter & STP används om ej annat angivits

Alfa 1-antichymotrypsin (symbol α1AC,[1] A1AC, or a1ACT) är ett glykoprotein av globulintyp som vandrar i alfaregionen vid proteinelektrofores. Den tillhör serinproteasinhibitorfamiljen (serpin). Hos människor kodas den av SERPINA3-genen.

Funktion

Alfa 1-antikymotrypsin hämmar aktiviteten hos vissa enzymer som kallas proteaser, såsom catepsin G som finns i neutrofiler och chymaser som finns i mastceller, genom att klyva dem till en annan form eller konformation. Denna aktivitet skyddar vissa vävnader, såsom nedre luftvägarna, från skador orsakade av proteolytiska enzymer.[2]

Detta protein produceras i levern och är ett akutfasprotein som induceras under inflammation.

Klinisk betydelse

Brist på alfa 1-antikymotrypsin har förknippats med leversjukdom. Mutationer har identifierats hos patienter med Parkinsons sjukdom och kronisk obstruktiv lungsjukdom.[3]

Alfa 1-antikymotrypsin är också förknippat med patogenesen av Alzheimers sjukdom eftersom det ökar bildandet av amyloidfibriller i denna sjukdom.[2]

Interaktioner

Alfa 1-antikymotrypsin har visat sig interagera med DNAJC1.[4]

Se även

  • Alfa-1 antitrypsin, en annan serpin som är analog för att skydda kroppen från överdrivna effekter av sina egna inflammatoriska proteaser.

Referenser

Den här artikeln är helt eller delvis baserad på material från engelskspråkiga Wikipedia, Alpha 1-antichymotrypsin, 27 mars 2022.

Noter

  1. ^ Logan, Carolynn M.; Rice, M. Katherine (1987). Logan's Medical and Scientific Abbreviations. Philadelphia: J. B. Lippincott Company. Sid. 3. ISBN 0-397-54589-4. https://archive.org/details/logansmedicalsci00loga/page/3. 
  2. ^ [a b] Kalsheker N (1996). ”Alpha 1-antichymotrypsin”. Int. J. Biochem. Cell Biol. 28 (9): sid. 961–4. doi:10.1016/1357-2725(96)00032-5. PMID 8930118. 
  3. ^ ”Entrez Gene: SERPINA3 serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 3”. https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=12. 
  4. ^ ”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): sid. 11432–43. March 2004. doi:10.1074/jbc.M310903200. PMID 14668352. 

Vidare läsning

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Akutfasproteiner
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