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TPP1 Lysosomal serine protease with tripeptidyl-peptidase I activity. May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases. Requires substrates with an unsubstituted N-terminus. Defects in TPP1 are the cause of neuronal ceroid lipofuscinosis type 2 (CLN2). A form of neuronal ceroid lipofuscinosis. Neuronal ceroid lipofuscinoses are progressive neurodegenerative, lysosomal storage diseases characterized by intracellular accumulation of autofluorescent liposomal material, and clinically by seizures, dementia, visual loss, and/or cerebral atrophy. The lipopigment pattern seen most often in CLN2 consists of curvilinear profiles. Belongs to the peptidase S53 family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: EC; Mitochondrial; Protease; Secreted; Secreted, signal peptide
Chromosomal Location of Human Ortholog: 11p15.4
Cellular Component: lysosomal lumen; lysosome
Molecular Function: endopeptidase activity; peptidase activity; peptide binding; protein binding; serine-type peptidase activity; tripeptidyl-peptidase activity
Biological Process: bone resorption; central nervous system development; epithelial cell differentiation; lipid metabolic process; lysosome organization and biogenesis; nervous system development; neuromuscular process controlling balance; peptide catabolic process; proteolysis
Disease: Ceroid Lipofuscinosis, Neuronal, 2
Reference #:  O14773 (UniProtKB)
Alt. Names/Synonyms: Cell growth-inhibiting gene 1 protein; ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease); CLN2; GIG1; growth-inhibiting protein 1; LPIC; lysosomal pepstatin insensitive protease; Lysosomal pepstatin-insensitive protease; MGC21297; TPP-1; TPP-I; TPP1; Tripeptidyl aminopeptidase; tripeptidyl peptidase I; Tripeptidyl-peptidase 1; Tripeptidyl-peptidase I
Gene Symbols: TPP1
Molecular weight: 61,248 Da
Basal Isoelectric point: 6.01  Predict pI for various phosphorylation states
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Protein Structure Not Found.
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