Anti -a1-Antitrypsin (AAT, alpha1 Proteinase Inhibitor, alpha1 PI, native and denatured)

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

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

Monoclonal

IgG1,k

6F5

Mouse

Recognizes human a-1 AT, native and denatured. Epitope specificity differs from that of clone 6F4. Reacts strongly with a-1 AT. Strong reaction is seen in ELISA with native and denatured a-1 AT directly coated onto the microtiter well. When tested in sandwich ELISA in combination with a polyclonal antibody against a-1 AT, it only recognizes native a-1 AT. In Western Blot after SDS-PAGE, this antibody reacts with native and denatured a-1 AT in reduced as well as non-reduced forms.

Affinity Purified
Purified by Protein A/G affinity chromatography.

Supplied as a liquid in PBS, pH 7.2, 15mM sodium azide, before the addition of glycerol to 40%.

May be stored at 4 degree C for short-term only. For long-term storage, aliquot and store at -20 degree C. Aliquots are stable for at least 12 months at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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.

The serum glycoprotein a1-antitrypsin (a-1 AT) is a typical member of the serpine family of serine protease inhibitors, and an inhibitor of the enzyme trypsin. a-1 AT is synthesized in the liver as a single polypeptide chain of approximately 51kD showing a marked degree of genetic polymorphism due to the presence of over 20 different alleles. Individuals homozygous for a-1 AT deficiency are predisposed to pulmonary emphysema and juvenile hepatic cirrhosis because of alterations in the amino acid and sialic acid components of the glycoprotein (1).

Antibodies; Abs to Coagulation Factors

ELISA (EL/EIA), Western Blot (WB)

Suitable for use in ELISA and Western Blot.
Dilution: ELISA: 1:7500

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, signal peptide; Secreted; Inhibitor

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

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