Tripeptidyl-peptidase I/TPP1

Tripeptidyl-peptidase I (TPP1), also known as CLN2, is a lysosomal serine protease detected in all tissues examined, with highest levels shown in heart and placenta. It is an aminopeptidase that cleaves tripeptides from the free N-terminus of polypeptides and may also have endopeptidase activity. Defects in TPP1 are the cause of classical late-infantile neuronal ceroid lipofusionosis, also known as ceroid lipofusionosis neuronal 2 (CLN2), a fatal childhood neurodegenerative disease characterized by progressive visual and mental decline, motor disturbance, epilepsy, and behavioral changes.