[5][6] Among other things it is heavily implicated in MHC (HLA) class-I processing, as it has both endopeptidase and exopeptidase activity.

[7] Biallelic deleterious variants in the TPP2 gene may result in a recessive disorder with immune deficiency, autoimmune disease and intellectual disability.

[8][9] Some genetic variants may result in a milder disease with sterile brain inflammation mimicking multiple sclerosis.

[10] These observations underline the fundamental role of TPP2 in cells of the immune system.

This article on a gene on human chromosome 13 is a stub.