Alfa 1-antitripsin
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| Inhibitor serpinske peptidaze, grupa A (alfa-1 antiproteinaza, antitripsin), član 1 | |||||||||||
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 Kristalna struktura serpina A1 (red) u inhibitornom kompleksu sa pankreatičkom elastazom (plavo). Prikaz je baziran na 2D26. | |||||||||||
| Dostupne strukture | |||||||||||
| 1ATU, 1D5S, 1EZX, 1HP7, 1IZ2, 1KCT, 1OO8, 1OPH, 1PSI, 1QLP, 1QMB, 2D26, 2QUG, 3CWL, 3CWM, 3DRM, 3DRU, 3NDD, 3NDF, 3NE4, 3T1P, 7API, 8API, 9API | |||||||||||
| Identifikatori | |||||||||||
| Simboli | SERPINA1; A1A; A1AT; AAT; PI; PI1; PRO2275; alpha1AT | ||||||||||
| Vanjski ID | OMIM: 107400 MGI: 891971 HomoloGene: 20103 GeneCards: SERPINA1 Gene | ||||||||||
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| Pregled RNK izražavanja | |||||||||||
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| Ortolozi | |||||||||||
| Vrsta | Čovek | Miš | |||||||||
| Entrez | 5265 | 20700 | |||||||||
| Ensembl | ENSG00000197249 | ENSMUSG00000066366 | |||||||||
| UniProt | P01009 | P07758 | |||||||||
| RefSeq (mRNA) | NM_000295 | NM_001252569 | |||||||||
| RefSeq (protein) | NP_000286 | NP_001239498 | |||||||||
| Lokacija (UCSC) | Chr 14: 94.84 - 94.86 Mb | Chr 12: 103.85 - 103.86 Mb | |||||||||
| PubMed pretraga | [1] | [2] | |||||||||
Alfa 1-antitripsin ili α1-antitripsin (A1AT) je proteazni inhibitor iz serpinske superfamilije. On je generalno poznat kao serumski tripsinski inhibitor. Alfa 1-antitripsin se takođe naziva alfa-1 proteinazni inhibitor (A1PI), jer inhibira širok opseg proteaza.[1] On štiti tkiva od enzima inflamatornih ćelija, posebno neutrofilne elastaze, i ima referentni opseg u krvi od 1,5 - 3,5 gram/litre, mada koncentracija može da bude znatno viša nakon akutne inflamacije.[2] U njegovom odsustvu, neutrofilna elastaza nesmetano razlaže elastin, koji doprinosi elastičnosti pluća, što stvara respiratorne probleme, kao što je emfisema.
Reference
Literatura
- Wu Y, Foreman RC (1991). „The molecular genetics of alpha 1 antitrypsin deficiency”. Bioessays 13 (4): 163–9. DOI:10.1002/bies.950130404. PMID 1859394.
- Kalsheker N (1989). „Alpha 1-antitrypsin: structure, function and molecular biology of the gene”. Biosci. Rep. 9 (2): 129–38. DOI:10.1007/BF01115992. PMID 2669992.
- Crystal RG (1990). „The alpha 1-antitrypsin gene and its deficiency states”. Trends Genet. 5 (12): 411–7. DOI:10.1016/0168-9525(89)90200-X. PMID 2696185.
- Carrell RW, Jeppsson JO, Laurell CB i dr.. (1982). „Structure and variation of human alpha 1-antitrypsin”. Nature 298 (5872): 329–34. DOI:10.1038/298329a0. PMID 7045697.
- Elliott PR, Abrahams JP, Lomas DA (1998). „Wild-type alpha 1-antitrypsin is in the canonical inhibitory conformation”. J. Mol. Biol. 275 (3): 419–25. DOI:10.1006/jmbi.1997.1458. PMID 9466920.
- Miyamoto Y, Akaike T, Maeda H (2000). „S-nitrosylated human alpha(1)-protease inhibitor”. Biochim. Biophys. Acta 1477 (1–2): 90–7. DOI:10.1016/S0167-4838(99)00264-2. PMID 10708851.
- Coakley RJ, Taggart C, O'Neill S, McElvaney NG (2001). „Alpha1-antitrypsin deficiency: biological answers to clinical questions”. Am. J. Med. Sci. 321 (1): 33–41. DOI:10.1097/00000441-200101000-00006. PMID 11202478.
- Lomas DA, Lourbakos A, Cumming SA, Belorgey D (2002). „Hypersensitive mousetraps, alpha1-antitrypsin deficiency and dementia”. Biochem. Soc. Trans. 30 (2): 89–92. DOI:10.1042/BST0300089. PMID 12023831.
- Kalsheker N, Morley S, Morgan K (2002). „Gene regulation of the serine proteinase inhibitors alpha1-antitrypsin and alpha1-antichymotrypsin”. Biochem. Soc. Trans. 30 (2): 93–8. DOI:10.1042/BST0300093. PMID 12023832.
- Perlmutter DH (2003). „Liver injury in α1-antitrypsin deficiency: an aggregated protein induces mitochondrial injury”. J. Clin. Invest. 110 (11): 1579–83. DOI:10.1172/JCI16787. PMC 151639. PMID 12464659.
- Lomas DA, Mahadeva R (2003). „α1-Antitrypsin polymerization and the serpinopathies: pathobiology and prospects for therapy”. J. Clin. Invest. 110 (11): 1585–90. DOI:10.1172/JCI16782. PMC 151637. PMID 12464660.
- Lisowska-Myjak B (2005). „AAT as a diagnostic tool”. Clin. Chim. Acta 352 (1–2): 1–13. DOI:10.1016/j.cccn.2004.03.012. PMID 15653097.
- Lomas DA (2005). „Molecular mousetraps, alpha1-antitrypsin deficiency and the serpinopathies”. Clinical medicine (London, England) 5 (3): 249–57. PMID 16011217.
- Rudnick DA, Perlmutter DH (2005). „Alpha-1-antitrypsin deficiency: a new paradigm for hepatocellular carcinoma in genetic liver disease”. Hepatology 42 (3): 514–21. DOI:10.1002/hep.20815. PMID 16044402.
- Mahr AD, Neogi T, Merkel PA (2006). „Epidemiology of Wegener's granulomatosis: Lessons from descriptive studies and analyses of genetic and environmental risk determinants”. Clin. Exp. Rheumatol. 24 (2 Suppl 41): S82–91. PMID 16859601.
- González-Sagrado M, López-Hernández S, Martín-Gil FJ, et al. (2000). "Alpha1-antitrypsin deficiencies masked by a clinical capillary electrophoresis system (CZE 2000)". Clinical Biochemistry, 33(1):79–80
Vanjske veze
Alfa 1-antitripsin na Wikimedijinoj ostavi
- Alpha 1-antitrypsin
- The MEROPS online database for peptidases and their inhibitors: I04.001 Arhivirano 2008-04-01 na Wayback Machine-u
- Proteopedia: Alpha-1-antitrypsin
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PDB Galerija
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