[3] This gene encodes a protein that is a member of the cysteine-aspartic acid protease (caspase) family.

Sequential activation of caspases plays a central role in the execution-phase of cell apoptosis.

Caspases exist as inactive proenzymes that undergo proteolytic processing at conserved aspartic residues to produce two subunits, large and small, that dimerize to form the active enzyme.

Mutations in this gene are associated with apoptosis defects seen in type II autoimmune lymphoproliferative syndrome.

Three alternatively spliced transcript variants encoding different isoforms have been described for this gene.