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Synonym: α1-Proteinase inhibitor, A1PI, AAT
MW: 52,000
Extinction Coefficient: 0.433
Lyophilized from 30 mM Na phosphate, pH 6.5, with 300 mM NaCl
Storage:
Purified Native Human Alpha 1 Antitrypsin, Human Plasma
Bulk Qty Available.
An acute-phase plasma protein found at 95-350 mg per 100 ml that functions as a protease inhibitor. Clinically, its deficiency is associated with two major diseases: pulmonary emphysema and early onset/juvenile hepatic cirrhosis. It is elevated in inflammatory conditions, malignancies, liver disease and pregnancy and also after surgical trauma and use of oral contraceptives. The simultaneous quantitative determination of alpha-1-PI and ceruloplasmin permits differential diagnosis of liver afflictions.
Activity: When tested with active-site titrated porcine pancreatic trypsin using Na-Benzoyl-L-Arginine-para-Nitroanilide Hydrochloride (L-BAPNA) as substrate, it is 75-100% inhibitory.
Purity: >=95% by SDS-PAGE
Prepared from plasma shown to be non reactive for HBsAg, anti-HCV, anti-HBc, and negative for anti-HIV 1 & 2 by FDA approved tests.
Lab products are laboratory reagents and are not to be administered to humans or used for any drug purpose.
For research use or for use in further manufacturing.
Product Citation:
Sinden, Nicola J., and Robert A. Stockley. "Proteinase 3 activity in sputum from subjects with alpha-1-antitrypsin deficiency and COPD."?European Respiratory Journal?41, no. 5 (2013): 1042-1050.
Borel F, Tang Q, Gernoux G, Greer C, Wang Z, et al.
Survival advantage of both human hepatocyte xenografts and genome edited hepatocytes for treatment of α-1 antitrypsin deficiency.
Molecular Therapy (2017), doi: 10.1016/j.ymthe.2017.09.020
Tang Q, Gruntman AM, Flotte TR.
Quantification of Total Human Alpha-1 Antitrypsin by Sandwich ELISA.
Methods Mol Biol. 2017;1639:211-216. doi: 10.1007/978-1-4939-7163-3_20.
Garratt LW, Sutanto EN, Ling KM, Looi K, et al.
Alpha-1 Antitrypsin Mitigates the Inhibition of Airway Epithelial Cell Repair by Neutrophil Elastase.
Am J Respir Cell Mol Biol. 2016 Mar;54(3):341-9. doi: 10.1165/rcmb.2015-0074OC.
Jansen BC, Falck D, de Haan N, Hipgrave Ederveen AL, Razdorov G, Lauc G, Wuhrer M.
LaCyTools: A Targeted Liquid Chromatography-Mass Spectrometry Data Processing Package for Relative Quantitation of Glycopeptides.
J Proteome Res. 2016 Jul 1;15(7):2198-210. doi: 10.1021/acs.jproteome.6b00171. Epub 2016 Jun 17.
Sinden NJ, et al.
α-1-Antitrypsin variants and the proteinase/antiproteinase imbalance in chronic obstructive pulmonary disease.
Am J Physiol Lung Cell Mol Physiol. 2015 Jan 15;308(2):L179-90.
Ramadass M, Ghebrehiwet B, Kew RR.
Enhanced recognition of plasma proteins in a non-native state by complement C3b. A possible clearance mechanism for damaged proteins in blood.
Mol Immunol. 2015 Mar;64(1):55-62. doi: 10.1016/j.molimm.2014.10.022. Epub 2014 Nov 15.
O'Dwyer CA, et al.
The BLT1 Inhibitory Function of α-1 Antitrypsin Augmentation Therapy Disrupts Leukotriene B4 Neutrophil Signaling.
J Immunol. 2015 Oct 15;195(8):3628-41. doi: 10.4049/jimmunol.1500038. Epub 2015 Sep 14.
Ungurs MJ, Sinden NJ, Stockley RA.
Progranulin is a substrate for neutrophil-elastase and proteinase-3 in the airway and its concentration correlates with mediators of airway inflammation in COPD
Am J Physiol Lung Cell Mol Physiol. 2014 Jan 1;306(1):L80-7. doi: 10.1152/ajplung.00221.2013. Epub 2013 Nov 1.
Sinden NJ, Koura F, Stockley RA.
The significance of the F variant of alpha-1-antitrypsin and unique case report of a PiFF homozygote.
BMC Pulm Med. 2014 Aug 7;14:132. doi: 10.1186/1471-2466-14-132.
Ramadass M, Ghebrehiwet B, Smith RJ, and Kew RR.
Generation of Multiple Fluid-Phase C3b:Plasma Protein Complexes during Complement Activation: Possible Implications in C3 Glomerulopathies.
J Immunol. 2014 Feb 1;192(3):1220-30. doi: 10.4049/jimmunol.1302288. Epub 2013 Dec 23.
