KEGG   Homo sapiens (human): 1200
Entry
1200              CDS       T01001                                 
Symbol
TPP1, CLN2, GIG1, LPIC, SCAR7, TPP-1
Name
(RefSeq) tripeptidyl peptidase 1
  KO
K01279  tripeptidyl-peptidase I [EC:3.4.14.9]
Organism
hsa  Homo sapiens (human)
Pathway
hsa04142  Lysosome
Disease
H00149  Neuronal ceroid lipofuscinosis
H01891  Autosomal recessive spinocerebellar ataxias
H02278  Jansky-Bielschowsky disease
Drug target
Cerliponase alfa: D10813<JP/US>
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    1200 (TPP1)
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors [BR:hsa01002]
    1200 (TPP1)
  09182 Protein families: genetic information processing
   03110 Chaperones and folding catalysts [BR:hsa03110]
    1200 (TPP1)
  09183 Protein families: signaling and cellular processes
   04147 Exosome [BR:hsa04147]
    1200 (TPP1)
Enzymes [BR:hsa01000]
 3. Hydrolases
  3.4  Acting on peptide bonds (peptidases)
   3.4.14  Dipeptidyl-peptidases and tripeptidyl-peptidases
    3.4.14.9  tripeptidyl-peptidase I
     1200 (TPP1)
Peptidases and inhibitors [BR:hsa01002]
 Serine peptidases
  Family S53
   1200 (TPP1)
Chaperones and folding catalysts [BR:hsa03110]
 Intramolecular chaperones
  Subtilisin family
   1200 (TPP1)
Exosome [BR:hsa04147]
 Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   1200 (TPP1)
SSDB
Motif
Pfam: Pro-kuma_activ Peptidase_S8
Other DBs
NCBI-GeneID: 1200
NCBI-ProteinID: NP_000382
OMIM: 607998
HGNC: 2073
Ensembl: ENSG00000166340
UniProt: O14773
Structure
Position
11:complement(6612768..6619422)
AA seq 563 aa
MGLQACLLGLFALILSGKCSYSPEPDQRRTLPPGWVSLGRADPEEELSLTFALRQQNVER
LSELVQAVSDPSSPQYGKYLTLENVADLVRPSPLTLHTVQKWLLAAGAQKCHSVITQDFL
TCWLSIRQAELLLPGAEFHHYVGGPTETHVVRSPHPYQLPQALAPHVDFVGGLHRFPPTS
SLRQRPEPQVTGTVGLHLGVTPSVIRKRYNLTSQDVGSGTSNNSQACAQFLEQYFHDSDL
AQFMRLFGGNFAHQASVARVVGQQGRGRAGIEASLDVQYLMSAGANISTWVYSSPGRHEG
QEPFLQWLMLLSNESALPHVHTVSYGDDEDSLSSAYIQRVNTELMKAAARGLTLLFASGD
SGAGCWSVSGRHQFRPTFPASSPYVTTVGGTSFQEPFLITNEIVDYISGGGFSNVFPRPS
YQEEAVTKFLSSSPHLPPSSYFNASGRAYPDVAALSDGYWVVSNRVPIPWVSGTSASTPV
FGGILSLINEHRILSGRPPLGFLNPRLYQQHGAGLFDVTRGCHESCLDEEVEGQGFCSGP
GWDPVTGWGTPNFPALLKTLLNP
NT seq 1692 nt   +upstreamnt  +downstreamnt
atgggactccaagcctgcctcctagggctctttgccctcatcctctctggcaaatgcagt
tacagcccggagcccgaccagcggaggacgctgcccccaggctgggtgtccctgggccgt
gcggaccctgaggaagagctgagtctcacctttgccctgagacagcagaatgtggaaaga
ctctcggagctggtgcaggctgtgtcggatcccagctctcctcaatacggaaaatacctg
accctagagaatgtggctgatctggtgaggccatccccactgaccctccacacggtgcaa
aaatggctcttggcagccggagcccagaagtgccattctgtgatcacacaggactttctg
acttgctggctgagcatccgacaagcagagctgctgctccctggggctgagtttcatcac
tatgtgggaggacctacggaaacccatgttgtaaggtccccacatccctaccagcttcca
caggccttggccccccatgtggactttgtggggggactgcaccgttttcccccaacatca
tccctgaggcaacgtcctgagccgcaggtgacagggactgtaggcctgcatctgggggta
accccctctgtgatccgtaagcgatacaacttgacctcacaagacgtgggctctggcacc
agcaataacagccaagcctgtgcccagttcctggagcagtatttccatgactcagacctg
gctcagttcatgcgcctcttcggtggcaactttgcacatcaggcatcagtagcccgtgtg
gttggacaacagggccggggccgggccgggattgaggccagtctagatgtgcagtacctg
atgagtgctggtgccaacatctccacctgggtctacagtagccctggccggcatgaggga
caggagcccttcctgcagtggctcatgctgctcagtaatgagtcagccctgccacatgtg
catactgtgagctatggagatgatgaggactccctcagcagcgcctacatccagcgggtc
aacactgagctcatgaaggctgccgctcggggtctcaccctgctcttcgcctcaggtgac
agtggggccgggtgttggtctgtctctggaagacaccagttccgccctaccttccctgcc
tccagcccctatgtcaccacagtgggaggcacatccttccaggaacctttcctcatcaca
aatgaaattgttgactatatcagtggtggtggcttcagcaatgtgttcccacggccttca
taccaggaggaagctgtaacgaagttcctgagctctagcccccacctgccaccatccagt
tacttcaatgccagtggccgtgcctacccagatgtggctgcactttctgatggctactgg
gtggtcagcaacagagtgcccattccatgggtgtccggaacctcggcctctactccagtg
tttggggggatcctatccttgatcaatgagcacaggatccttagtggccgcccccctctt
ggctttctcaacccaaggctctaccagcagcatggggcaggactctttgatgtaacccgt
ggctgccatgagtcctgtctggatgaagaggtagagggccagggtttctgctctggtcct
ggctgggatcctgtaacaggctggggaacacccaacttcccagctttgctgaagactcta
ctcaacccctga

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