Gosset Molecular Classification
Serpin superfamily, Glycoprotein
Other Names
α1-antitrypsin, A1AT, alpha-1 proteinase inhibitor, Alpha( 1–proteinase inhibitor), Alpha( 1-antiproteinase), Alpha( 1PI), Alpha( 1AP)
Disease Roles
Alpha-1-antitrypsin Overview
Alpha-1-antitrypsin (AAT) is a serine protease inhibitor primarily synthesized in the liver. It protects tissues, especially in the lungs, from damage by neutrophil elastase. Deficiency or dysfunction leads to lung and liver diseases. Therapeutic AAT preparations are used to treat severe deficiency.
Mechanism of Action
Suicide substrate inhibition via RCL insertion/conformational trapping; irreversible inhibition of target proteases like neutrophil elastase.
Biological Functions
Serine protease inhibitor
Neutrophil elastase inhibitor
Immunomodulator
Regulation of inflammation
Tissue protection
Regulation of chemotaxis
Disease Associations
COPD
Emphysema
Liver disease
Cirrhosis
Safety Considerations
- Risk of allergic reactions to therapeutic AAT products
- Potential for transmission of infectious agents with plasma-derived products
- Ineffectiveness of AAT augmentation therapy in certain patient subgroups
Interacting Drugs
Aralast NP®
Associated Biomarkers
| Biomarker |
|---|
| AAT serum levels for diagnosis of deficiency |
| SERPINA1 gene variants for risk assessment |