Tripeptidyl-peptidase 1 (UniProt: O14773; also known as TPP1, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, LPIC, Tripeptidyl aminopeptidase) is encoded by the TPP1 (also known as CLN2, GIG1, UNQ267/PRO304) gene (Gene ID: 1200) in human. TPP1 is a lysosomal serine protease that acts as a non-specific peptidase and sequentially removes tripeptides from the free N termini of small polypeptides produced by lysosomal proteinases. TPP1 is expressed in several tissues, with tissues associated with peptide hormone production displaying higher level of expression. Human TPP I is synthesized as a zymogen with an apparent mass of 68 kDa, which is processed to a 48 kDa mature enzyme by removal of 179 amino acid prodomain. TPP I zymogen has five potential N-glycosylation sites at amino acid positions 210, 222, 286, 313, and 443. The N-linked glycans can add around 10 kDa to the molecular mass of TPP 1. Like many other lysosomal hydrolases, TPP I proenzyme is able to autoactivate in the acidic pH in vitro. Mutations in TPP1 gene are shown to be a causative factor in ceroid lipofuscinosis, neuronal, 2 (CLN2) a progressive neurodegenerative, lysosomal storage disease, characterized by intracellular accumulation of autofluorescent liposomal material and clinically manifested seizures, dementia, visual loss, and/or cerebral atrophy.
Synonyms: Tripeptidyl-peptidase 1, Cell growth-inhibiting gene 1 protein, Lysosomal pepstatin-insensitive protease, LPIC, Tripeptidyl aminopeptidase
Application: Research CategoryNeuroscience
Other Notes: Concentration: Please refer to lot specific datasheet.