a1-Antitrypsin, Protein

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 14; NC_000014.8 (94843084..94857029, complement). Location: 14q32.1

Human plasma

Highly Purified
0.95

Supplied lyophilized from 0.02M ammonium carbonate buffer. May contain traces of buffer salts.Reconstitute with sterile phosphate buffer pH7.0, 0.15m sodium chloride.

May be stored at 4 degree C. For long-term storage, aliquot and store at 4 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of SERPINA1 protein vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Alpha-1-antitrypsin (AAT) consisting of a single polypeptide chain and has a molecular weight of 51kDa. It exists in a number of genetic variants. MM variant is the most common. Alpha-1-AT is synthesized in the liver and it acts as an inhibitor of proteases such as trypsin, elastase, chymotrypsin, collagenase, leukocytic proteases, plasmin, and thrombin, which may be released during inflammatory reactions in the lung. In the absence of AAT, these enzymes are not inhibited and they may digest pulmonary parenchyma. AAT deficiency is associated with chronic obstructive lung disease (emphysema) and less frequently with hepatic cirrhosis in infants and respiratory distress of the newborn. Increase in AAT occurs as an acute phase response to tissue necrosis and inflammation. Serum level of alpha-1-AT is elevated in rheumatoid arthritis, bacterial infections, vasculitis, and carcinomatosis. In colorectal and lung carcinomas, AAT has also related to their invasive and metastatic capacity. The average level of AAT in plasma is 1.3g/L.

Molecular Biology; MB-Coagulation Factors

46,737 Da[Similar Products]

serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1

alpha-1-antitrypsin; serpin A1; alpha-1-antiproteinase; alpha-1-antitrypsin null; alpha-1 protease inhibitor; protease inhibitor 1 (anti-elastase), alpha-1-antitrypsin; serine (or cysteine) proteinase inhibitor, clade A, member 1

Alpha-1-antitrypsin

AAT; PI; SPAAT  [Similar Products]

A1AT_HUMAN

The protein encoded by this gene is secreted and is a serine protease inhibitor whose targets include elastase, plasmin, thrombin, trypsin, chymotrypsin, and plasminogen activator. Defects in this gene can cause emphysema or liver disease. Several transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]

SERPINA1: Inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. Irreversibly inhibits trypsin, chymotrypsin and plasminogen activator. The aberrant form inhibits insulin-induced NO synthesis in platelets, decreases coagulation time and has proteolytic activity against insulin and plasmin. Defects in SERPINA1 are the cause of alpha-1-antitrypsin deficiency (A1ATD). A disorder whose most common manifestation is emphysema, which becomes evident by the third to fourth decade. A less common manifestation of the deficiency is liver disease, which occurs in children and *****s, and may result in cirrhosis and liver failure. Environmental factors, particularly cigarette smoking, greatly increase the risk of emphysema at an earlier age. Belongs to the serpin family. 3 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted; Inhibitor; Secreted, signal peptide

Chromosomal Location of Human Ortholog: 14q32.1

Cellular Component: Golgi apparatus; proteinaceous extracellular matrix; extracellular space; endoplasmic reticulum; extracellular region

Molecular Function: serine-type endopeptidase inhibitor activity; identical protein binding; protein binding; protease binding; glycoprotein binding

Biological Process: platelet activation; response to chromate; platelet degranulation; response to triglyceride; response to cytokine stimulus; response to lead ion; acute-phase response; response to hypoxia; response to methanol; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus

Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.