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Alpha 1-antitrypsin
Alpha 1-antitrypsin (A1AT) is type of protein produced
mainly in the liver that inhibits the actions of numerous
enzymes (e.g., trypsin) that promote the breakdown of
certain proteins. These types of enzymes (types of
proteins that help produce chemical reactions) are
known as proteases. The liver is the largest organ in
the body, one role of which is to produces important
chemicals (such as A1AT) for certain bodily functions.
Trypsin is not produced in the liver but is produced in
the pancreas. The pancreas is a long organ behind the
stomach that produces several types of hormones and
helps digest food with pancreatic juices that contains
enzymes. Hormones are natural chemicals produced by
the body that are released into the blood and have a
specific effect on bodily tissues.
Structure of alpha 1-antitrypsin
 
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A1AT plays an important role in protecting tissues from the enzymes of cells that cause
inflammation, which is why A1AT levels increase in response to inflammation. One such
enzyme that it protects the body from is neutrophil elastase, which can disrupt
connective tissue.

A rare form of A1AT inhibits thrombin, which is an enzyme that plays an important role in
blood clotting.

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There are 25 different forms of the alpha-antitrypsin gene. Genes
are units of material contained in a personís cells that contain
coded instructions as for how certain bodily characteristics will
develop.

If someone has two identical forms of the alpha-antitrypsin gene,
this will lead to abnormal production of A1AT, which means that it
will not function properly. Thus, there will be a natural deficiency of
A1AT activity in the body. A1AT deficiency is one of the most
commonly inherited genetic disorders among Caucasians.
Without enough A1AT, the body is not properly protected from the effects of inflammation. Although most people with A1AT deficiency will not develop clinically significant disease, more severe cases can be associated with early-onset emphysema and liver disease. Emphysema is an abnormality characterized by enlargement and damage of the walls of tiny balloon-like sacs in the lungs that air travels to. This can occur due to decreased A1AT activity in the lungs. This is because without A1AT, elastase can accumulate in the lung tissue and damage the tissue that helps absorb oxygen. Liver disease in A1AT is caused by deposits of excessive amounts of abnormal A1AT in the liver, which causes scarring and dysfunction. A1AT is considered a type of antitrypsin, meaning it is a substance that inhibits trypsin, a type of enzyme that helps with digestion. Antitryptic and antitrypsic means pertaining to properties of antitrypsin. A1AT is also known as alpha1 trypsin inhibitor, human alpha-1 proteinase inhibitor (A1P1), and serum trypsin inhibitor.