SERPINA1 (Alpha-1-antitrypsin) variants and mutations

SERPINA1 (also known as Alpha-1-antitrypsin) is a human protein-coding gene encoding an alpha-1-antitrypsin protein. Its circulating alpha-1-antitrypsin activity protects lung tissue by neutralizing neutrophil elastase and related proteases. Severe deficiency predisposes to early emphysema, while accumulation of misfolded protein in hepatocytes can cause chronic liver disease. This analysis covers 951 SERPINA1 variants and mutations. Of these, 79% have computational variant effect predictions. Disease context includes Alpha-1-antitrypsin deficiency, chronic obstructive pulmonary disease, and alpha 1-antitrypsin deficiency. Example SERPINA1 variants include M1V, P2A, and P2L.

Variant analysis overview

Variant and mutation evidence

Clinical, disease, and population context

Protein structure and variant hotspots

Data sources

Evidence in this analysis draws on EBI Proteins Variation, UniProt, gnomAD v4, EuropePMC, 3D Hotspot Analysis, Interaction Network Analysis, Protein Data Bank, AlphaFold DB, gnomAD constraint, Open Targets, ClinGen, MaveDB, LitVar.

Notable SERPINA1 variants

Examples include M1V, P2A, P2L, P2Q, P2S, S3C, S3F, S3Y. Listed records include available protein-change notation, database identifiers, clinical classifications, computational predictions, population evidence, experimental measurements, and disease context.