Description
Alpha-1 Antitrypsin or α1-antitrypsin (A1AT) is a protease inhibitor belonging to the serpin superfamily. It is generally known as serum trypsin inhibitor. Alpha 1-antitrypsin is also referred to as alpha-1 proteinase inhibitor (A1PI) because it inhibits a wide variety of proteases. It protects tissues from enzymes of inflammatory cells, especially neutrophil elastase, and has a reference range in blood of 1.5-3.5 gram/liter (in US the reference range is generally expressed as mg/dL or micromoles), but the concentration can rise manyfold upon acute inflammation. In its absence, neutrophil 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 adults and cirrhosis in adults or children.
Abbr
A1AT, Native (Human)
Alias
A1A; A1AT; AAT; PI1
Pathway
Complement and Coagulation Cascades, organism-specific biosystem; Complement and coagulation cascades, organism-specific biosystem; Complement and coagulation cascades, conserved biosystem; FOXA1 transcription factor network, organism-specific biosystem; Hemostasis, organism-specific biosystem; Platelet activation, signaling and aggregation, organism-specific biosystem; Platelet degranulation, organism-specific biosystem
Function
protease binding; protein binding; serine-type endopeptidase inhibitor activity; serine-type endopeptidase inhibitor activity
Synonyms
Alpha-1-Antitrypsin; A1AT; α1-antitrypsin; SERPINA1; A1A; A1AT; AAT; PI; PI1; PRO2275; alpha1AT
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