Mouse monoclonal antibody to human TPP-1 [3B1]: IgG

TPP1 is a lysosomal serine protease with tripeptidyl-peptidase I activity. It may act as a non-specific lysosomal peptidase which releases an N-terminal tripeptide from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. TPP1 is detected in lysosomes and melanosomes in all tissues but it is highest in the heart and placenta.A TPP1 is activated by autocatalytic proteolytic processing upon acidification within lysosomes. Defects in TPP1 cause classical late-infantile neuronal ceroid lipofuscinosis (LINCL) which is also known as ceroid lipofuscinosis 2 (CLN2). It is a fatal childhood neurodegenerative disease characterised by progressive visual and mental deterioration, motor disturbance, epilepsy and behavioural changes.