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Protein Page:
TPP1 (human)
p Phosphorylation
ac Acetylation
me Methylation
m1 Mono-methylation
m2 Di-methylation
m3 Tri-methylation
ub Ubiquitylation
sm Sumoylation
ne Neddylation
gl O-GlcNAc
ga O-GalNAc
pa Palmitoylation
ad Adenylation
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; EC 3.4.14.9; Protease; Secreted
Chromosomal Location of Human Ortholog: 11p15
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
Select Structure to View Below

TPP1

Protein Structure Not Found.
Download PyMol Script
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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



 LTP 

LTP: 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.


 HTP 

HTP: 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
0 1 Q373 WSVSGRHQFRPTFPA
0 1 Y459‑p VAALSDGyWVVSNRV
0 1 T478‑p VSGTSAStPVFGGIL
  mouse

 
T200 VSLHLGVTPSVLRQR
K345‑ac RVNTEFMkAAARGLT
K372 WSVSGRHKFRPSFPA
Y458 VAALSDGYWVVSNMV
T477 VSGTSASTPVFGGIL
  rat

 
T201 VGLHLGVTPSVLRQR
K346 RVNTEFMKAAARGLT
K373‑ac WSVSGRHkFRPSFPA
Y459 VAALSDGYWVVSNMV
T478 VSGTSASTPVFGGIL
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