AAH14863.1
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
34,464 Da
NCBI Official Full Name
Tripeptidyl peptidase I
NCBI Official Synonym Full Names
tripeptidyl peptidase 1
NCBI Protein Information
tripeptidyl-peptidase 1
UniProt Protein Name
Tripeptidyl-peptidase 1
UniProt Synonym Protein Names
Cell growth-inhibiting gene 1 protein; Lysosomal pepstatin-insensitive protease; LPIC; Tripeptidyl aminopeptidase; Tripeptidyl-peptidase I; TPP-I
UniProt Synonym Gene Names
NCBI Summary for TPP1
This gene encodes a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity. It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome. [provided by RefSeq, Jul 2008]
UniProt Comments for TPP1
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.
Protein type: EC 3.4.14.9; 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
Research Articles on TPP1
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Products associated with anti-TPP1 antibody
Pathways associated with anti-TPP1 antibody
Diseases associated with anti-TPP1 antibody
Organs/Tissues associated with anti-TPP1 antibody
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