Features Interactions Isoform Disease Linear motifs Fingerprint Network All partners

TPP1_HUMAN

Lysosomal serine protease with tripeptidyl-peptidase I activity (PubMed, PubMed, PubMed). May act as a non-specific lysosomal peptidase which generates tripeptides from the breakdown products produced by lysosomal proteinases (PubMed, PubMed, PubMed). Requires substrates with an unsubstituted N-terminus (PubMed). [View more on UniProt]

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Download full PS network for entry.
Position Amino acid Mutation Disease Overlap with binding region
365 Cys Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
365 Cys Tyr Ceroidlipofuscinosis,neuronal,2(CLN2) -
447 Arg His Ceroidlipofuscinosis,neuronal,2(CLN2) -
77 Gly Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
206 Arg Cys Ceroidlipofuscinosis,neuronal,2(CLN2) -
287 Ile Asn Ceroidlipofuscinosis,neuronal,2(CLN2) -
343 Glu Lys Ceroidlipofuscinosis,neuronal,2(CLN2) -
385 Val Asp Ceroidlipofuscinosis,neuronal,2(CLN2) -
389 Gly Glu Ceroidlipofuscinosis,neuronal,2(CLN2) -
422 Gln His Ceroidlipofuscinosis,neuronal,2(CLN2) -
454 Ala Glu Ceroidlipofuscinosis,neuronal,2(CLN2) -
475 Ser Leu Ceroidlipofuscinosis,neuronal,2(CLN2) -
127 Arg Gln Ceroidlipofuscinosis,neuronal,2(CLN2) -
153 Ser Pro Ceroidlipofuscinosis,neuronal,2(CLN2) -
206 Arg His Ceroidlipofuscinosis,neuronal,2(CLN2) -
277 Val Met Ceroidlipofuscinosis,neuronal,2(CLN2) -
278 Gln Pro Ceroidlipofuscinosis,neuronal,2(CLN2) -
284 Gly Val Ceroidlipofuscinosis,neuronal,2(CLN2) -
286 Asn Ser Ceroidlipofuscinosis,neuronal,2(CLN2) -
353 Thr Pro Ceroidlipofuscinosis,neuronal,2(CLN2) -
428 Lys Asn Ceroidlipofuscinosis,neuronal,2(CLN2) -
473 Gly Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
481 Phe Cys Ceroidlipofuscinosis,neuronal,2(CLN2) -
482 Gly Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
202 Pro Leu Ceroidlipofuscinosis,neuronal,2(CLN2) -
544 Pro Ser Ceroidlipofuscinosis,neuronal,2(CLN2) -
62 Ser Thr Ceroidlipofuscinosis,neuronal,2(CLN2) -
209 Tyr His Ceroidlipofuscinosis,neuronal,2(CLN2) -
266 Arg Gln Ceroidlipofuscinosis,neuronal,2(CLN2) -
339 Arg Gln Ceroidlipofuscinosis,neuronal,2(CLN2) -
382 Ser Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
448 Ala Val Ceroidlipofuscinosis,neuronal,2(CLN2) -
501 Gly Cys Ceroidlipofuscinosis,neuronal,2(CLN2) -
504 Asn Tyr Ceroidlipofuscinosis,neuronal,2(CLN2) -
548 Trp Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -
466 Val Gly Spinocerebellarataxia,autosomalrecessive,7(SCAR7) -
278 Gln Arg Ceroidlipofuscinosis,neuronal,2(CLN2) -

No annotated instance was found. To search for linear motifs, use the ELM prediction server.

Molecular function

Term Name % Distance from the top of the tree Annotated in TPP1_HUMAN
GO:0046872 metal ion binding 40.5405 4 yes
GO:0015399 primary active transmembrane transporter activity 29.7297 4 no
GO:0005524 ATP binding 21.6216 5 no
GO:0017076 purine nucleotide binding 21.6216 4 no
GO:0030554 adenyl nucleotide binding 21.6216 5 no
GO:0032555 purine ribonucleotide binding 21.6216 4 no
GO:0032559 adenyl ribonucleotide binding 21.6216 5 no
GO:0035639 purine ribonucleoside triphosphate binding 21.6216 4 no
GO:0004175 endopeptidase activity 10.8108 4 yes
GO:0004252 serine-type endopeptidase activity 8.1081 5 yes
GO:0008236 serine-type peptidase activity 8.1081 4 yes
GO:0008238 exopeptidase activity 5.4054 4 yes
GO:0008240 tripeptidyl-peptidase activity 5.4054 6 yes
GO:0070008 serine-type exopeptidase activity 5.4054 5 yes
GO:0120146 sulfatide binding 5.4054 4 yes

Biological process

Term Name % Distance from top the of the tree Annotated in TPP1_HUMAN
GO:0031323 regulation of cellular metabolic process 37.8378 4 no
GO:0048523 negative regulation of cellular process 37.8378 4 no
GO:0060255 regulation of macromolecule metabolic process 37.8378 4 no
GO:0048522 positive regulation of cellular process 35.1351 4 no
GO:0010646 regulation of cell communication 32.4324 4 no
GO:0051171 regulation of nitrogen compound metabolic process 32.4324 4 no
GO:0080090 regulation of primary metabolic process 32.4324 4 no
GO:0008104 protein localization 29.7297 4 yes
GO:0051246 regulation of protein metabolic process 29.7297 5 no
GO:0009893 positive regulation of metabolic process 29.7297 4 no
GO:0006996 organelle organization 27.0270 4 yes
GO:0022607 cellular component assembly 27.0270 4 no
GO:0010243 response to organonitrogen compound 27.0270 4 no
GO:0009966 regulation of signal transduction 27.0270 4 no
GO:0022900 electron transport chain 24.3243 4 no
GO:0009892 negative regulation of metabolic process 24.3243 4 no
GO:0010605 negative regulation of macromolecule metabolic process 24.3243 5 no
GO:0051172 negative regulation of nitrogen compound metabolic process 24.3243 5 no
GO:0010468 regulation of gene expression 24.3243 5 no
GO:0010941 regulation of cell death 24.3243 4 no
GO:0043067 regulation of programmed cell death 24.3243 5 no
GO:0048585 negative regulation of response to stimulus 24.3243 4 no
GO:0009060 aerobic respiration 21.6216 6 no
GO:0015980 energy derivation by oxidation of organic compounds 21.6216 4 no
GO:0022904 respiratory electron transport chain 21.6216 5 no
GO:0045333 cellular respiration 21.6216 5 no
GO:0019220 regulation of phosphate metabolic process 21.6216 6 no
GO:0031324 negative regulation of cellular metabolic process 21.6216 5 no
GO:0031399 regulation of protein modification process 21.6216 6 no
GO:0051174 regulation of phosphorus metabolic process 21.6216 5 no
GO:0043933 protein-containing complex organization 21.6216 4 no
GO:0009889 regulation of biosynthetic process 21.6216 4 no
GO:0009894 regulation of catabolic process 21.6216 4 no
GO:0010648 negative regulation of cell communication 21.6216 5 no
GO:0023057 negative regulation of signaling 21.6216 4 no
GO:0031325 positive regulation of cellular metabolic process 21.6216 5 no
GO:0031329 regulation of cellular catabolic process 21.6216 5 no
GO:0042981 regulation of apoptotic process 21.6216 6 no
GO:1902531 regulation of intracellular signal transduction 21.6216 5 no
GO:0006508 proteolysis 16.2162 4 yes
GO:0009057 macromolecule catabolic process 16.2162 4 yes
GO:1901565 organonitrogen compound catabolic process 16.2162 4 yes
GO:0030163 protein catabolic process 13.5135 4 yes
GO:0033365 protein localization to organelle 13.5135 5 yes
GO:0006518 peptide metabolic process 8.1081 4 yes
GO:0007033 vacuole organization 8.1081 5 yes
GO:0007417 central nervous system development 8.1081 4 yes
GO:0043603 cellular amide metabolic process 8.1081 4 yes
GO:0001894 tissue homeostasis 5.4054 5 yes
GO:0007039 protein catabolic process in the vacuole 5.4054 5 yes
GO:0007040 lysosome organization 5.4054 7 yes
GO:0007399 nervous system development 5.4054 4 yes
GO:0030855 epithelial cell differentiation 5.4054 4 yes
GO:0034502 protein localization to chromosome 5.4054 6 yes
GO:0043171 peptide catabolic process 5.4054 4 yes
GO:0045453 bone resorption 5.4054 6 yes
GO:0050885 neuromuscular process controlling balance 5.4054 5 yes
GO:0050905 neuromuscular process 5.4054 4 yes
GO:0060249 anatomical structure homeostasis 5.4054 4 yes
GO:0070198 protein localization to chromosome, telomeric region 5.4054 7 yes
GO:0080171 lytic vacuole organization 5.4054 6 yes
GO:1905146 lysosomal protein catabolic process 5.4054 6 yes

Disease

Term Name % Distance from top the of the tree Annotated in TPP1_HUMAN
DOID:1289 neurodegenerative disease 8.1081 4 yes
DOID:331 central nervous system disease 8.1081 3 yes
DOID:0050753 cerebellar ataxia 5.4054 6 yes
DOID:0050950 autosomal recessive cerebellar ataxia 5.4054 7 yes
DOID:0050951 hereditary ataxia 5.4054 5 yes
DOID:0080059 autosomal recessive spinocerebellar ataxia 7 5.4054 8 yes
DOID:0110726 neuronal ceroid lipofuscinosis 2 5.4054 6 yes
DOID:14503 neuronal ceroid lipofuscinosis 5.4054 5 yes
DOID:3211 lysosomal storage disease 5.4054 3 yes
DOID:9455 lipid storage disease 5.4054 4 yes
Uniprot ID Details Highest evidence Localizing into PSD HPA (protein expression in neurons)
POTE1_HUMAN [view interactions] Low throughput no no
STN1_HUMAN [view interactions] Low throughput no no
CTC1_HUMAN [view interactions] Low throughput no no
TERT_HUMAN [view interactions] Low throughput no no
RNF8_HUMAN [view interactions] Low throughput no yes
UBP7_HUMAN [view interactions] Low throughput no yes
TINF2_HUMAN [view interactions] Low throughput no no
TPP1_HUMAN [view entry] [view interactions] Low throughput yes yes
GLUT4_HUMAN [view interactions] High throughput no no
MBD2_HUMAN [view interactions] High throughput no yes
GRDN_HUMAN [view interactions] High throughput no no
NALP2_HUMAN [view interactions] High throughput no no
ELK1_HUMAN [view interactions] High throughput no yes
HD_HUMAN [view entry] [view interactions] High throughput yes yes
PKN3_HUMAN [view interactions] High throughput no yes
PRIO_HUMAN [view entry] [view interactions] High throughput yes yes
BCAR1_HUMAN [view interactions] High throughput no yes
HSC20_HUMAN [view interactions] High throughput no yes
ANGI_HUMAN [view interactions] High throughput no no
TX101_HUMAN [view interactions] High throughput no no
CEP55_HUMAN [view interactions] High throughput no yes
FBX42_HUMAN [view interactions] High throughput no yes
FBXL4_HUMAN [view interactions] High throughput no yes
WWP2_HUMAN [view interactions] High throughput no no
TRAF4_HUMAN [view interactions] High throughput no yes
CISH_HUMAN [view interactions] High throughput no yes
ZBT38_HUMAN [view interactions] High throughput no no
RN183_HUMAN [view interactions] High throughput no no
OTUB1_HUMAN [view interactions] High throughput no yes
MYC_HUMAN [view interactions] High throughput no yes
ESR1_HUMAN [view interactions] High throughput no no
A2MG_HUMAN [view interactions] High throughput no yes
TOM20_HUMAN [view entry] [view interactions] High throughput yes yes
FBX6_HUMAN [view interactions] High throughput no yes
CFTR_HUMAN [view interactions] High throughput no no
EGFR_HUMAN [view interactions] High throughput no no
ABCE1_HUMAN [view interactions] High throughput no yes
MCM2_HUMAN [view interactions] High throughput no no
MPIP3_HUMAN [view interactions] High throughput no yes
OMA1_HUMAN [view interactions] High throughput no no
NDKM_HUMAN [view interactions] High throughput no yes
GDC_HUMAN [view interactions] High throughput no no
RPTOR_HUMAN [view interactions] High throughput no yes
EGLN3_HUMAN [view interactions] High throughput no no
ESR2_HUMAN [view interactions] High throughput no no
RECQ4_HUMAN [view interactions] High throughput no yes
MAGF1_HUMAN [view entry] [view interactions] High throughput yes yes
NHLC2_HUMAN [view interactions] High throughput no yes
APEX1_HUMAN [view interactions] High throughput no yes
CUL7_HUMAN [view interactions] High throughput no yes
WDR5_HUMAN [view interactions] High throughput no no
KMCP1_HUMAN [view interactions] High throughput no no
DGKG_HUMAN [view interactions] High throughput no no
TSN2_HUMAN [view interactions] High throughput no no
POP5_HUMAN [view interactions] High throughput no no
DHB8_HUMAN [view interactions] High throughput no yes
BASI_HUMAN [view entry] [view interactions] High throughput yes no
TM192_HUMAN [view interactions] High throughput no yes
XRN2_HUMAN [view interactions] High throughput no yes
BRCA1_HUMAN [view interactions] High throughput no yes
CALX_HUMAN [view entry] [view interactions] High throughput yes yes
CCD47_HUMAN [view interactions] High throughput no yes
OGT1_HUMAN [view entry] [view interactions] High throughput yes yes
DNJA1_HUMAN [view entry] [view interactions] High throughput yes yes
HNRH1_HUMAN [view entry] [view interactions] High throughput yes yes
NDUA9_HUMAN [view entry] [view interactions] High throughput yes yes
ODBB_HUMAN [view interactions] High throughput no yes
PON2_HUMAN [view interactions] High throughput no yes
QCR1_HUMAN [view entry] [view interactions] High throughput yes yes
LPPRC_HUMAN [view entry] [view interactions] High throughput yes yes
NCPR_HUMAN [view entry] [view interactions] High throughput yes yes
HXK1_HUMAN [view entry] [view interactions] High throughput yes yes
NNTM_HUMAN [view entry] [view interactions] High throughput yes yes
VATA_HUMAN [view entry] [view interactions] High throughput yes yes
NDUS1_HUMAN [view entry] [view interactions] High throughput yes no
IDHP_HUMAN [view entry] [view interactions] High throughput yes yes
P4HA1_HUMAN [view interactions] High throughput no yes
AT2A2_HUMAN [view entry] [view interactions] High throughput yes yes
QCR2_HUMAN [view entry] [view interactions] High throughput yes yes
NDUS3_HUMAN [view entry] [view interactions] High throughput yes yes
NDUV1_HUMAN [view entry] [view interactions] High throughput yes yes
HCDH_HUMAN [view entry] [view interactions] High throughput yes yes
VATB2_HUMAN [view entry] [view interactions] High throughput yes yes
LRC59_HUMAN [view entry] [view interactions] High throughput yes yes
GAL3A_HUMAN [view interactions] High throughput no yes
GAL3B_HUMAN [view interactions] High throughput no yes
LONM_HUMAN [view entry] [view interactions] High throughput yes yes
APOE_HUMAN [view entry] [view interactions] High throughput yes yes
GSTP1_HUMAN [view entry] [view interactions] High throughput yes no
NDUS7_HUMAN [view entry] [view interactions] High throughput yes no
ASPH_HUMAN [view interactions] High throughput no yes
LTOR2_HUMAN [view interactions] High throughput no yes
COR1C_HUMAN [view entry] [view interactions] High throughput yes yes
TERA_HUMAN [view entry] [view interactions] High throughput yes yes
TFR1_HUMAN [view interactions] High throughput no yes
ATPG_HUMAN [view interactions] High throughput no yes
ATPB_HUMAN [view interactions] High throughput no yes
ETFD_HUMAN [view entry] [view interactions] High throughput yes yes
ADAS_HUMAN [view interactions] High throughput no yes
NDUS8_HUMAN [view interactions] High throughput no yes
EMC4_HUMAN [view interactions] High throughput no yes
CY1_HUMAN [view entry] [view interactions] High throughput yes yes
SPRE_HUMAN [view interactions] High throughput no yes
THTM_HUMAN [view interactions] High throughput no yes
COR1B_HUMAN [view entry] [view interactions] High throughput yes no
CYB5_HUMAN [view interactions] High throughput no no
MCCB_HUMAN [view entry] [view interactions] High throughput yes yes
NDUBB_HUMAN [view interactions] High throughput no yes
BGAL_HUMAN [view interactions] High throughput no yes
ARL8B_HUMAN [view interactions] High throughput no yes
SQSTM_HUMAN [view interactions] High throughput no yes
CLN5_HUMAN [view interactions] Computational no yes
CLN3_HUMAN [view interactions] Computational no no
TERF1_HUMAN [view interactions] Computational no no
CLN6_HUMAN [view interactions] Computational no no
MFSD8_HUMAN [view interactions] Computational no yes
XBP1_HUMAN [view interactions] Computational no no