Tripeptidyl peptidase I and its role in neurodegenerative and tumor diseases

Abstract

Tripeptidyl peptidase I (TPPI) is a lysosomal enzyme widely distributed inmammals and humans. Its genetically determined deficiency causes the classicallate-infantile form of neuronal ceroid lipofuscinosis, a fatal hereditary neurodegenerativedisease associated with severe symptoms and early death, usually inthe second decade of life. Many studies also show that TPPI is differentiallyregulated under various pathological conditions such as malignancy, neurodegeneration, ischemia, and inflammation, pointing at possible enzyme involvementin the pathogeneses of these entities. This chapter focuses on the TPPIparticipation in neurodegenerative and neoplastic diseases.