84-9364-10　Recombinant Human SerpinA1/A1AT Protein （His Tag）（Active） 100ug　PKSH031695

特徴

• 背景：SerpinA1, also known as Alpha-1 antitrypsin （AAT）, is a prototype member of the Serpin superfamily of the serine protease inhibitors.
• This serine protease inhibitor blocks the protease, neutrophil elastase.
• Alpha-1 antitrypsin is mainly produced in the liver and acts as an antiprotease.
• Its principal function is to inactivate neutrophil elastase, preventing tissue damage.
• SerpinA1 （alpha1-antitrypsin）, an acute phase protein and the classical neutrophil elastase inhibitor, is localized within lipid rafts in primary human monocytes in vitro.
• It association with monocytes is inhibited by cholesterol depleting/efflux-stimulating agents （nystatin, filipin, MbetaCD （methyl-beta-cyclodextrin） and oxidized low-density lipoprotein （oxLDL） and conversely, enhanced by free cholesterol.
• Furthermore, SerpinA1/monocyte association per se depletes lipid raft cholesterol as characterized by the activation of extracellular signal-regulated kinase 2, formation of cytosolic lipid droplets, and a complete inhibition of oxLDL uptake by monocytes.
• Previous population studies have suggested that heterozygote status for the AAT gene （SerpinA1） is a risk factor for chronic rhinosinusitis with nasal polyposis （CRSwNP）.
• Alpha-1 antitrypsin deficiency is a recently identified genetic disease that occurs almost as frequently as cystic fibrosis.
• It is caused by various mutations in the SerpinA1 gene, and has numerous clinical implications.
• Alpha-1 antitrypsin deficiency is an inherited disease affecting the lung and liver.
• In the liver, alpha-1 antitrypsin deficiency may manifest as benign neonatal hepatitis syndrome; a small percentage of adults develop liver fibrosis, with progression to cirrhosis and hepatocellular carcinoma.
• Its most important physiologic functions are the protection of pulmonary tissue from aggressive proteolytic enzymes and regulation of pulmonary immune processes.