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Protein Page:
TPP1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitination
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylylation
sn S-Nitrosylation
ca Caspase cleavage
sc Succinylation

Overview
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: Secreted, signal peptide; Mitochondrial; Protease; Secreted; EC 3.4.14.9
Cellular Component: lysosomal lumen; mitochondrion; lysosome; melanosome
Molecular Function: peptidase activity; tripeptidyl-peptidase activity; protein binding; serine-type peptidase activity; metal ion binding; serine-type endopeptidase activity; endopeptidase activity; peptide binding
Biological Process: cell death; nervous system development; cellular protein metabolic process; unfolded protein response, activation of signaling protein activity; epithelial cell differentiation; unfolded protein response; lysosome organization and biogenesis; peptide catabolic process; protein catabolic process; lipid metabolic process; neuromuscular process controlling balance; proteolysis; bone resorption
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
Select Structure to View Below

TPP1

Protein Structure Not Found.


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Modification Sites and Domains Show Modification Legend
Click here to view phosphorylation modifications only

Modification Sites in Parent Protein, Orthologs, and Isoforms Show Modification Legend
 

Show Multiple Sequence Alignment


 SS 

SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.


 MS 

MS: The number of records in which this modification site was assigned using ONLY proteomic discovery-mode mass spectrometry.


       human

 
1 1 T201-p VGLHLGVtPSVIRKR
0 1 K346 RVNTELMKAAARGLT
  mouse

 
T200 VSLHLGVTPSVLRQR
K345-ac RVNTEFMkAAARGLT
  rat

 
T201 VGLHLGVTPSVLRQR
K346 RVNTEFMKAAARGLT
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