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Alpha-1 antitrypsin (AAT)
- Target
- AAT
- Molecular classification
- Serine protease inhibitor, Serpin
01
Overview
Alpha-1 antitrypsin (AAT) is a serine protease inhibitor primarily produced in the liver. Its main function is to protect tissues, especially in the lungs, from damage by proteolytic enzymes such as neutrophil elastase. Deficiency in AAT leads to unopposed activity of neutrophil elastase, resulting in tissue damage and diseases such as emphysema and liver cirrhosis.
- Other names
- alpha-1 proteinase inhibitoralpha-1 protease inhibitorSerpin family member
02
Mechanism of action
Inhibits neutrophil elastase and other serine proteases by forming a stable complex, preventing tissue degradation.
03
Biological functions
Proteolytic enzyme inhibitionInflammation controlTissue protection
04
Disease associations
COPDEmphysemaLiver cirrhosisHepatocellular carcinomaPanniculitis
05
Safety considerations
Risk of emphysema/COPD in deficiencyRisk of liver disease in deficiencyImpact of smoking on AAT activityPotential for misfolding and aggregation in deficiency
06
Biomarkers
AAT serum levelsSERPINA1 genotype
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