protein

Human Alpha 1-Antitrypsin

MBS318173

1 mg

195 USD

Native Protein

Human Alpha 1-Antitrypsin

Alpha 1 Antitrypsin

An acute phase plasma protein found at 290mg per 100ml that functions as a protease inhibitor.

alpha-1-antitrypsin; Alpha-1-antitrypsin; alpha-1-antitrypsin; serpin A1; OTTHUMP00000197150; OTTHUMP00000197151; OTTHUMP00000197152; OTTHUMP00000197153; OTTHUMP00000197154; alpha-1-antiproteinase; 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

SERPINA1; SERPINA1; PI; A1A; AAT; PI1; A1AT; MGC9222; PRO2275; MGC23330; alpha1AT; AAT; PI

A1AT_HUMAN

Human Plasma

Alpha 1 Antitrypsin

>95% pure (SDS-PAGE)

Purified, Lyophilized. Reconstitute with 222ul distilled water.

1.9mg/ml (prior to lyophilization)

Store at -20 degree C. Avoid multiple freeze/thaw cycles.

Inactivation: Not applicable.

Buffer: Lyophilized from 30mM Sodium phosphate, pH 6.5 containing 300mM Sodium chloride. Preservative: No. Lyophilized: Yes. Warnings: All human source materials have tested negative for HIV 1 and HIV 2 antibodies and non-reactive for anti-HCV, anti-HBc and HBsAg. No test guarantees a product to be non-infectious. Therefore, all material derived from human fluids or tissues sho

Purified Proteins and Bioactive Peptides

177831

P01009

46,737 Da

Complement And Coagulation Cascades Pathway 198880!!Complement And Coagulation Cascades Pathway 83073!!Complement And Coagulation Cascades Pathway 484!!FOXA1 Transcription Factor Network Pathway 137979!!Formation Of Platelet Plug Pathway 106029!!Hemostasis Pathway 106028!!Platelet Activation Pathway 106034!!Platelet Degranulation Pathway 106050!!Response To Elevated Platelet Cytosolic Ca2+ Pathway 106048

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]

Function: 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. Ref.17 Ref.18 Ref.24Short peptide from AAT (SPAAT) is a reversible chymotrypsin inhibitor. It also inhibits elastase, but not trypsin. Its major physiological function is the protection of the lower respiratory tract against proteolytic destruction by human leukocyte elastase (HLE). Ref.17 Ref.18 Ref.24. Subcellular location: Secreted Ref.24. Short peptide from AAT: Secreted extracellular space extracellular matrix Ref.24. Tissue specificity: Plasma. Domain: The reactive center loop (RCL) extends out from the body of the protein and directs binding to the target protease. The protease cleaves the serpin at the reactive site within the RCL, establishing a covalent linkage between the carboxyl group of the serpin reactive site and the serine hydroxyl of the protease. The resulting inactive serpin-protease complex is highly stable. Post-translational modification: Several isomers are observed, resulting from the combination of different N-linked glycan structures and mature N-terminus. N-linked glycan at Asn-107 is alternatively di-antennary, tri-antennary or tetra-antennary, whereas glycan at Asn-70 is di-antennary with trace amounts of tri-antennary, and glycan at Asn-271 is exclusively di-antennary. The structure of the antennas is Neu5Ac(alpha1-6)Gal(beta1-4)GlcNAc attached to the core structure Man(alpha1-6)[Man(alpha1-3)]Man(beta1-4)GlcNAc(beta1-4)GlcNAc. Some antennas are fucosylated, which forms a Lewis-X determinant.Proteolytic processing may yield the truncated form that ranges from Asp-30 to Lys-418. Polymorphism: The sequence shown is that of the M1V allele which is the most common form of PI (44 to 49%). Other frequent alleles are: M1A 20 to 23%; M2 10 to 11%; M3 14 to 19%. Involvement in disease: Defects in SERPINA1 are the cause of alpha-1-antitrypsin deficiency (A1ATD) [. MIM:613490]. 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. Ref.58 Ref.60 Ref.62. Miscellaneous: The aberrant form is found in the plasma of chronic smokers, and persists after smoking is ceased. It can still be found ten years after smoking has ceased. Sequence similarities: Belongs to the serpin family. Sequence caution: The sequence CAD62334.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.The sequence CAD62585.1 differs from that shown. Reason: Erroneous initiation. Translation N-terminally shortened.

1 mg

195 USD