Alpha-1 Antitrypsin

Alpha-1 Antitrypsin

Target · Serine protease inhibitor

Alpha-1 Antitrypsin Alpha-1 antitrypsin · serpin family A member 1

Alpha-1 Antitrypsin (AAT, also known as A1AT, alpha1AT, A1A, PI1 and serpin family A member 1, encoded by SERPINA1) is a secreted serine protease inhibitor of the serpin family. Its major physiological role is protecting the lower respiratory tract from proteolytic destruction by human leukocyte elastase. Browse Assay Genie's current range of Alpha-1 Antitrypsin research reagents below, or read on for the biology behind the target.

Secreted serpinInhibits leukocyte elastaseLinked to emphysema and liver disease
418 aa
Protein length
~47 kDa
Molecular weight
14q32.13
Gene locus
Serpin
Protein family
Target information

What is Alpha-1 Antitrypsin (serpin family A member 1)?

Alpha-1 Antitrypsin is a reversible chymotrypsin inhibitor that 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). NCBI Gene describes the protein as a serine protease inhibitor of the serpin superfamily whose targets include elastase, plasmin, thrombin, trypsin, chymotrypsin and plasminogen activator.

The SERPINA1 gene encodes the Alpha-1 Antitrypsin protein, which belongs to the serpin family and sits in the HGNC gene group of serpin peptidase inhibitors. The protein is found in the extracellular space and extracellular matrix, and is also located in the endoplasmic reticulum. It is produced in the liver, the bone marrow, by lymphocytic and monocytic cells in lymphoid tissue, and by the Paneth cells of the gut. Several transcript variants have been described, and all encode the same protein. The rat ortholog is Serpina1.

Defects in the SERPINA1 gene are associated with chronic obstructive pulmonary disease, emphysema and chronic liver disease, which makes Alpha-1 Antitrypsin a frequent subject in respiratory and hepatic research. The gene was previously named serine (or cysteine) proteinase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1, and the symbol PI is also used in older literature. Researchers studying AAT often use an Alpha-1 Antitrypsin antibody or ELISA kit to measure the protein in experimental systems.

Respiratory disease research

Alpha-1 Antitrypsin protects the lower respiratory tract from leukocyte elastase, and SERPINA1 defects are associated with chronic obstructive pulmonary disease and emphysema.

Liver disease studies

Defects in the SERPINA1 gene are associated with chronic liver disease, and the protein is produced in the liver.

Protease inhibition biology

As a serpin, Alpha-1 Antitrypsin inhibits targets including elastase, plasmin, thrombin, chymotrypsin and plasminogen activator, making it a model for serine protease regulation.

Immune and gut tissue biology

Expression by lymphocytic and monocytic cells in lymphoid tissue and by gut Paneth cells supports study of the protein beyond the liver.

Background

Alpha-1 Antitrypsin frequently asked questions

What does Alpha-1 Antitrypsin do?

Alpha-1 Antitrypsin is a reversible chymotrypsin inhibitor that also inhibits elastase but not trypsin. Its major physiological function is protecting the lower respiratory tract from proteolytic destruction by human leukocyte elastase. It belongs to the serpin family of serine protease inhibitors.

What other names is Alpha-1 Antitrypsin known by?

The protein is also called AAT, A1AT, alpha1AT, A1A, PI1 and PI. HGNC names the gene SERPINA1, serpin family A member 1, previously serine (or cysteine) proteinase inhibitor, clade A (alpha-1 antiproteinase, antitrypsin), member 1. Several transcript variants encode the same protein.

Which diseases are linked to the SERPINA1 gene?

NCBI Gene reports that defects in SERPINA1 are associated with chronic obstructive pulmonary disease, emphysema and chronic liver disease. A rat ortholog, Serpina1, is recorded, so check the datasheet of any product for its validated reactivity.

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Related targets

Other members of the SERPINA serpin peptidase inhibitor group, related to Alpha-1 Antitrypsin.

Gene and protein annotations from HGNC, NCBI Gene and UniProt P01009; product specifications from Assay Genie datasheets.

Alpha-1 Antitrypsin Product listing