antibody

Sheep anti-human alpha1Antitrypsin (alpha1AT), Peroxidase Conjugated Affinity-Purified IgG

MBS512005

0.1 mg

310 USD

polyclonal

sheep

HRP

Antibody

Sheep anti-human alpha1Antitrypsin (alpha1AT), Peroxidase Conjugated Affinity-Purified IgG

alpha1-Antitrypsin

alpha1-Antitrypsin, human

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

Polyclonal

Sheep

Prior to conjugation this antibody was specific for alpha1AT as demonstrated by immunoelectrophoresis and ELISA.

Affinity purified, peroxidase conj. IgG. Vial containing 0.1ml of affinity-purified IgG conjugated to horseradish peroxidase (HRP) through carbohydrate groups. Total protein is 0.1 mg.

APIgG-HRP conjugates as a clear, slightly red-brown liquid.

APIgG-HRP concentration is mg/ml, determined by absorbance using an extinction coefficient (E1%280) of 14.

Store between -10 and -20 degree C. Product will become viscous but will not freeze. Avoid storage in frost-free freezers. Keep vial tightly capped. Allow product to warm to room temperature and gently mix before use. Avoid exposure to sodium azide as this is an inhibitor of peroxidase activity.

Suitable as a source of peroxidase-labeled antibodies to alpha1AT.

Immunogen: Human alpha1antitrypsin purified from plasma. Rz Ratio (Reinheitszahl, A 403 /A 280</sub):: 0.456 as determined spectrophotometrically.

Buffer: A buffered stabilizer solution containing 50% (v/v) glycerol.

alpha1Antitrypsin (alpha1AT), also known as alpha1Proteinase inhibitor (alpha1PI), is the most abundant protease inhibitor in blood and a member of the SERPIN family of proteinase inhibitors. Serum levels are typically 1.3 mg/ml (25 uM) but alpha1AT is an acute phase protein and concentrations can rise four-fold during inflammatory episodes or tissue injury. Low levels in circulation have been associated with pulmonary disease such as emphysema. alpha1AT is a single chain molecule with a mass of 52,000 daltons that is produced primarily in the liver and to a lesser extent by blood monocytes and intestinal epithelium. Based on association rates, the primary target enzyme for alpha1AT is believed to be neutrophil elastase1,2, but alpha1AT is a broadspectrum inhibitor for many serine proteinases and the main role of alpha1AT in vivo is likely that of a "backup" inhibitor and proteinase scavenger in fluids and tissues. Although the association rates of alpha1AT with other enzymes are lower, the high concentration in plasma makes it an important inhibitor of activated Protein C, activated FXI, thrombin and plasmin1-4. Enzyme inhibition by alpha1AT occurs through proteolytic cleavage between Met358 and Ser359, which induces a conformational change in alpha1AT locking the enzyme into a stable, inactive 1:1 enzyme-inhibitor complex.

1. Johnson D, Travis J; Oxidative Inactivation of Human alpha-1-Proteinase Inhibitor; JBC 254, pp4022-4026, 1979. 2. Travis J, Johnson D; Human alpha-1-Proteinase Inhibitor; Methods in Enzymology, 80, pp 754-765, 1981. 3. Heeb MJ, Griffin JH; Physiologic Inhibition of Human Activated Protein C by alpha-1-Trypsin Inhibitor; JBC 263, pp1163-11616, 1988. 4. Scott CF, Schapira M, James HL, Cohen AB, Colman RW; The Inactivation of Factor XIa by plasma protease inhibitors: Predominant role of alpha1protease inhibitor and protective effect of high molecular weight kininogen. J Clin Invest 69, pp 844, 1982.

1703025

P01009.3

P01009

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.

0.1 mg

310 USD