Alpha-1-Antitrypsin, Human Plasma | MyBioSource MBS135723 product information

product summary

company name :

MyBioSource

product type :

protein

product name :

Alpha-1-Antitrypsin, Human Plasma

catalog :

MBS135723

quantity :

5 mg

price :

380 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS135723

products type :

Protein

products full name :

Alpha-1-Antitrypsin, Human Plasma

products short name :

Alpha-1-Antitrypsin, Human Plasma

other names :

alpha-1-antitrypsin; Alpha-1-antitrypsin; 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; serpin peptidase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1; Alpha-1 protease inhibitor; Alpha-1-antiproteinase; Serpin A1

products gene name :

SERPINA1

other gene names :

SERPINA1; SERPINA1; PI; A1A; AAT; PI1; A1AT; PRO2275; alpha1AT; AAT; PI; SPAAT

uniprot entry name :

A1AT_HUMAN

host :

Human plasma

sequence length :

418

purity :

>95% by SDS-PAGE analysis

form :

Lyophilized powder

concentration :

Lyophilized

storage stability :

Store at -70 degree C. Shelf Life: 3 years from delivery

other info1 :

Volume: 2.17 ml before lyophilization . Specific Activity: 75-100% inhibitory when tested with active-site titrated porcine pancreatic trypsin using N?-Benzoyl-L-Arginine-para-Nitroanilide Hydrochloride (L-BAPNA) as substrate.

other info2 :

Buffer: 0.03M Sodium Phosphate; 0.3M NaCl; pH 6.5. Extinction Coefficient: 0.433

products categories :

Plasma Proteins; Human Plasma Proteins

products description :

An acute-phase plasma protein found at 290 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-I-PI and ceruloplasmin permits differential diagnosis of liver, afflictions. Per the WHO collaborative study establishing the first International Alpha-I Antitrypsin Standard and Cowden 01, Fisher GE and Weeks RL 2005 ? A pilot study comparing the purity, functionality and isoform composition of alpha-I proteinase inhibitor (human) products?, Current Medical Research and Opinions 21,887-883, we have changed the extinction coefficient for determining alpha-l antitrypsin concentration from 5.3 (A280, 1 percent solution, I cm path length) to 4.33 (A280, 1 percent solution, 1 cm path length). Add deionized water to original volume, aliquot and freeze unused portion.

ncbi gi num :

50363217

ncbi acc num :

NP_000286.3

ncbi gb acc num :

NM_000295.4

uniprot acc num :

P01009

ncbi mol weight :

52,000

ncbi pathways :

Complement And Coagulation Cascades Pathway (198880); Complement And Coagulation Cascades Pathway (83073); Complement And Coagulation Cascades Pathway (484); FOXA1 Transcription Factor Network Pathway (137979); Hemostasis Pathway (106028); Platelet Activation, Signaling And Aggregation Pathway (106034); Platelet Degranulation Pathway (106050); Response To Elevated Platelet Cytosolic Ca2+ Pathway (106048)

ncbi summary :

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]

uniprot summary :

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 adults, 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: Inhibitor; Secreted; 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; response to methanol; response to hypoxia; acute-phase response; response to lipopolysaccharide; blood coagulation; response to estradiol stimulus. Disease: Alpha-1-antitrypsin Deficiency; Pulmonary Disease, Chronic Obstructive

size1 :

5 mg

price1 :

380 USD

size2 :

10 mg

price2 :

580

more info or order :

MyBioSource product webpage