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

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

Polyclonal

IgG

polyclonal

Rabbit

Alpha-1-Antitrypsin is a purified immunoglobulin fraction of rabbit antiserum that is filter sterilized and diluted in buffer pH 7.5, containing BSA and sodium azide as a preservative.

Store at 2 to 8 degree C in the dark.

Manufactured in an ISO 13485:2016 Certified Laboratory.

Small volumes of anti-SERPINA1 antibody 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 (A1AT) is a glycoprotein generally known as serum trypsin inhibitor. Alpha-1-Antitrypsin is also referred to as alpha-1 proteinase inhibitor (A1PI) because it is a serine protease inhibitor (serpin), inhibiting a wide variety of proteases. It protects tissues from enzymes of infl ammatory cells, especially elastase, and has a reference range in blood of 1.5 – 3.5 gram/liter (in the U.S. the reference range is generally expressed as mg/dL or micromoles), but the concentration can rise many fold upon acute infl ammation. In its absence, elastase is free to break down elastin, which contributes to the elasticity of the lungs, resulting in respiratory complications such as emphysema, or COPD (Chronic Obstructive Pulmonary Disease) in *****s and cirrhosis in *****s or children. Alpha-1-Antitrypsin is considered to be very useful in the study of inherited AAT defi ciency, benign and Malignant Hepatic Tumors and Yolk-Sac Carcinomas. Positive staining for A-1-Antitrypsin may also be used in detection of benign and malignant lesions of a histiocytic nature. Sensitivity and specifi city of the results have made this antibody a useful tool in the screening of patients with Cryptogenic Cirrhosis or other forms of liver disease with portal fi brosis of unknown etiology.

46,737 Da[Similar Products]

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

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

Alpha-1-antitrypsin

AAT; PI??[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]

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 *****s, 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.

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