alpha 1 Antitrypsin Rabbit Polyclonal Antibody

Function

Alpha 1-Antitrypsin is a inhibitor of serine proteases. Its primary target is elastase, but it also has a moderate affinity for plasmin and thrombin. It 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.

Background References

1. "Canonical inhibitor-like interactions explain reactivity of alpha1-proteinase inhibitor Pittsburgh and antithrombin with proteinases."Dementiev A., Simonovic M., Volz K., Gettins P.G.J. Biol. Chem. 278:37881-37887(2003)

2. "A novel alpha-1-antitrypsin r281del variant found in a population sample from the Basque country."Seixas S., Garcia O., Amorim A., Rocha J.Hum. Mutat. 15:121-122(2000)

Sequence Similarity

Belongs to the serpin family.

Tissue Specificity

Ubiquitous. Expressed in leukocytes and plasma.

Post-translational Modification

N-glycosylated. Differential glycosylation produces a number of isoforms. N-linked glycan at Asn-107 is alternatively di-antennary, tri-antennary or tetra-antennary. The glycan at Asn-70 is di-antennary with trace amounts of tri-antennary. Glycan at Asn-271 is exclusively di-antennary. Structure of glycans at Asn-70 and Asn-271 is Hex5HexNAc4. The structure of the antennae 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 antennae are fucosylated, which forms a Lewis-X determinant.; Proteolytic processing may yield the truncated form that ranges from Asp-30 to Lys-418.; (Microbial infection) Proteolytically processed by Staphylococcus aureus seryl, cysteinyl, and metallo-proteases.

Subcellular Location

Secreted

UNIPROT

SwissProt: P01009 Human

Synonyms