KEGG   ORTHOLOGY: K01279
Entry
K01279                      KO                                     
Symbol
TPP1, CLN2
Name
tripeptidyl-peptidase I [EC:3.4.14.9]
Pathway
map04142  Lysosome
Disease
H00149  Neuronal ceroid lipofuscinosis
H01891  Autosomal recessive spinocerebellar ataxias
H02278  Jansky-Bielschowsky disease
Brite
KEGG Orthology (KO) [BR:ko00001]
 09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    K01279  TPP1, CLN2; tripeptidyl-peptidase I
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors
    K01279  TPP1, CLN2; tripeptidyl-peptidase I
  09182 Protein families: genetic information processing
   03110 Chaperones and folding catalysts
    K01279  TPP1, CLN2; tripeptidyl-peptidase I
  09183 Protein families: signaling and cellular processes
   04147 Exosome
    K01279  TPP1, CLN2; tripeptidyl-peptidase I
Enzymes [BR:ko01000]
 3. Hydrolases
  3.4  Acting on peptide bonds (peptidases)
   3.4.14  Dipeptidyl-peptidases and tripeptidyl-peptidases
    3.4.14.9  tripeptidyl-peptidase I
     K01279  TPP1, CLN2; tripeptidyl-peptidase I
Peptidases and inhibitors [BR:ko01002]
 Serine peptidases
  Family S53
   K01279  TPP1, CLN2; tripeptidyl-peptidase I
Chaperones and folding catalysts [BR:ko03110]
 Intramolecular chaperones
  Subtilisin family
   K01279  TPP1, CLN2; tripeptidyl-peptidase I
Exosome [BR:ko04147]
 Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   K01279  TPP1, CLN2; tripeptidyl-peptidase I
Genes
HSA: 1200(TPP1)
PTR: 450999(TPP1)
PPS: 100969179(TPP1)
GGO: 101149790(TPP1)
PON: 100440001(TPP1)
NLE: 100586648(TPP1)
HMH: 116476575(TPP1)
MCC: 709838(TPP1)
MCF: 101867272(TPP1)
MNI: 105488695(TPP1)
CSAB: 103241292(TPP1)
CATY: 105575039(TPP1)
PANU: 101006485(TPP1)
TGE: 112606671(TPP1)
MLEU: 105533898(TPP1)
RRO: 104678305(TPP1)
RBB: 108543236(TPP1)
TFN: 117081025(TPP1)
PTEH: 111524649(TPP1)
CANG: 105520548(TPP1)
CJC: 100895900(TPP1)
SBQ: 101030033(TPP1)
CIMI: 108288319(TPP1)
CSYR: 103257949(TPP1)
MMUR: 105883443(TPP1)
LCAT: 123642366(TPP1)
PCOQ: 105818381(TPP1)
OGA: 100959054(TPP1)
MMU: 12751(Tpp1)
MCAL: 110298569(Tpp1)
MPAH: 110319059(Tpp1)
RNO: 83534(Tpp1)
MCOC: 116102738(Tpp1)
ANU: 117710198(Tpp1)
MUN: 110559588(Tpp1)
CGE: 100759190(Tpp1)
MAUA: 101843832(Tpp1)
PROB: 127228102(Tpp1)
PLEU: 114683811(Tpp1)
MORG: 121457527(Tpp1)
MFOT: 126494111
AAMP: 119817670(Tpp1)
NGI: 103737566(Tpp1)
HGL: 101707185(Tpp1)
CPOC: 100733945(Tpp1)
CCAN: 109696314(Tpp1)
DORD: 106000379(Tpp1)
DSP: 122117653(Tpp1)
PLOP: 125361199(Tpp1)
NCAR: 124958585
MMMA: 107135610(Tpp1)
OCU: 100356773
OPI: 101517413(TPP1)
TUP: 102474485(TPP1)
GVR: 103597707(TPP1)
CFA: 485337(TPP1)
CLUD: 112667557(TPP1)
VVP: 112910866(TPP1)
VLG: 121475975(TPP1)
NPO: 129505126(TPP1)
AML: 100473391(TPP1)
UMR: 103681402(TPP1)
UAH: 113245816(TPP1)
UAR: 123782735(TPP1)
ELK: 111159341
LLV: 125078220
MPUF: 101675115(TPP1)
MNP: 132023468(TPP1)
MLK: 131808646(TPP1)
NVS: 122912678(TPP1)
ORO: 101364552(TPP1)
EJU: 114219328(TPP1)
ZCA: 113915227(TPP1)
MLX: 118004087(TPP1)
NSU: 110576529(TPP1)
LWW: 102740770(TPP1)
FCA: 101093296(TPP1)
PYU: 121021124(TPP1)
PCOO: 112865484(TPP1)
PBG: 122483500(TPP1)
PVIV: 125176249(TPP1)
LRUF: 124505273
PTG: 102968271(TPP1)
PPAD: 109271080(TPP1)
PUC: 125936730
AJU: 106981175
HHV: 120221444(TPP1)
BTA: 515575(TPP1)
BOM: 102279785(TPP1)
BIU: 109569865(TPP1)
BBUB: 102403898(TPP1)
BBIS: 104987992(TPP1)
CHX: 102176981(TPP1)
OAS: 101116858(TPP1)
BTAX: 128060387(TPP1)
ODA: 120876542(TPP1)
CCAD: 122449495(TPP1)
MBEZ: 129554579(TPP1)
SSC: 100623352(TPP1)
CFR: 102512212(TPP1)
CBAI: 105076828(TPP1)
CDK: 105097984(TPP1)
VPC: 102538740(TPP1)
BACU: 103017792(TPP1)
BMUS: 118899902(TPP1)
LVE: 103084335(TPP1)
OOR: 101288758(TPP1)
DLE: 111169170(TPP1)
PCAD: 102995041(TPP1)
PSIU: 116758264(TPP1)
NASI: 112407683(TPP1)
ECB: 100070095(TPP1)
EPZ: 103553536(TPP1)
EAI: 106838179(TPP1)
MYB: 102249131(TPP1)
MYD: 102763994(TPP1)
MMYO: 118664764(TPP1)
MLF: 102420099(TPP1)
PKL: 118712965(TPP1)
EFUS: 103302787(TPP1)
MNA: 107540509(TPP1)
DRO: 112312796(TPP1)
SHON: 118990810(TPP1)
AJM: 119041209(TPP1)
PDIC: 114500407(TPP1)
PHAS: 123819213(TPP1)
MMF: 118616782(TPP1)
PPAM: 129076406(TPP1)
HAI: 109376530(TPP1)
RFQ: 117030579(TPP1)
PALE: 102887010(TPP1)
PGIG: 120590231(TPP1)
PVP: 105309083(TPP1)
RAY: 107504378(TPP1)
MJV: 108384234(TPP1)
TOD: 119255545(TPP1)
SARA: 101542279(TPP1)
SETR: 126018154(TPP1)
LAV: 100666542(TPP1)
TMU: 101346186
ETF: 101653844(TPP1)
DNM: 101429403(TPP1)
MDO: 100030970(TPP1)
GAS: 123240474(TPP1)
SHR: 100918462(TPP1)
AFZ: 127555378
PCW: 110200515(TPP1)
OAA: 103167781(TPP1)
GGA: 425576(TPP1)
PCOC: 116239524(TPP1)
MGP: 104916731(TPP1)
CJO: 107308570(TPP1)
TPAI: 128088601(TPP1)
LMUT: 125695685(TPP1)
NMEL: 110391738(TPP1)
APLA: 101792345(TPP1)
ACYG: 106049774(TPP1)
CATA: 118259799(TPP1)
AFUL: 116502288(TPP1)
TGU: 100232321(TPP1)
LSR: 110474958(TPP1)
SCAN: 103824409(TPP1)
PMOA: 120499970(TPP1)
OTC: 121334227
PRUF: 121355144(TPP1)
GFR: 102035657(TPP1)
FAB: 101810332(TPP1)
OMA: 130248400(TPP1)
PHI: 102114063(TPP1)
PMAJ: 107198426(TPP1)
CCAE: 111943727(TPP1)
CCW: 104698105(TPP1)
ETL: 114069900
ZAB: 102062982(TPP1)
ACHL: 103809824(TPP1)
SVG: 106861771(TPP1)
MMEA: 130582734(TPP1)
HRT: 120749462(TPP1)
FPG: 101920803(TPP1)
FCH: 102051334(TPP1)
CLV: 102094093
EGZ: 104130616(TPP1)
NNI: 104014881(TPP1)
PCRI: 104029187(TPP1)
PLET: 104628454(TPP1)
PCAO: 104053372(TPP1)
TALA: 116961971(TPP1)
PADL: 103924871(TPP1)
AFOR: 103901145(TPP1)
ACHC: 115353221(TPP1)
HLE: 104837588(TPP1)
AGEN: 126048322
GCL: 127026232
CCRI: 104157404(TPP1)
BREG: 104634407(TPP1)
FGA: 104083605(TPP1)
GSTE: 104264258(TPP1)
MNB: 103772866(TPP1)
OHA: 104328432(TPP1)
NNT: 104406087(TPP1)
SHAB: 115602342(TPP1)
DPUB: 104302340(TPP1)
CPEA: 104385372
CVF: 104284862(TPP1)
RTD: 128909835(TPP1)
TEO: 104376113(TPP1)
AAM: 106497748
AROW: 112964918(TPP1)
NPD: 112955053(TPP1)
TGT: 104580295(TPP1)
DNE: 112993503(TPP1)
ASN: 102377067(TPP1)
AMJ: 102560569(TPP1)
CPOO: 109305562(TPP1)
GGN: 109302196(TPP1)
PSS: 102459935
CMY: 102933063(TPP1)
CCAY: 125638755(TPP1)
DCC: 119860201(TPP1)
CPIC: 101951787(TPP1)
TST: 117871333(TPP1)
CABI: 116818179(TPP1)
MRV: 120399176(TPP1)
ACS: 100556940(tpp1)
ASAO: 132772355(TPP1)
PVT: 110078112(TPP1)
PBI: 103059366(TPP1)
PMUR: 107288659(TPP1)
CTIG: 120310033(TPP1)
TSR: 106541898
PGUT: 117670878(TPP1)
APRI: 131199219(TPP1)
PTEX: 113450417(TPP1)
NSS: 113426067(TPP1)
VKO: 123022455(TPP1)
PMUA: 114595159(TPP1)
PRAF: 128412415(TPP1)
ZVI: 118084890(TPP1)
HCG: 128350066(TPP1)
GJA: 107116951(TPP1)
STOW: 125431778(TPP1)
EMC: 129325454(TPP1)
XLA: 414505(tpp1.L)
XTR: 100124868(tpp1)
NPR: 108799885(TPP1)
RTEM: 120928863(TPP1)
BBUF: 120996656(TPP1)
BGAR: 122932330(TPP1)
MUO: 115468239(TPP1)
GSH: 117362881(TPP1)
DRE: 798347(tpp1)
SGH: 107594810
PTET: 122361712(tpp1)
LROH: 127179676(tpp1)
OMC: 131523129(tpp1)
PPRM: 120477347(tpp1)
RKG: 130100728(tpp1)
MAMB: 125268908(tpp1)
CIDE: 127498869
TROS: 130560242(tpp1)
TDW: 130436540(tpp1)
MANU: 129417562(tpp1)
IPU: 108270113
IFU: 128613171(tpp1)
PHYP: 113538931(tpp1)
SMEO: 124390242(tpp1)
TFD: 113659646(tpp1)
TVC: 132852722(tpp1)
AMEX: 103045803(tpp1)
CMAO: 118823512(tpp1)
EEE: 113576644(tpp1)
CHAR: 105899641(tpp1)
TRU: 101071259(tpp1)
TFS: 130540194(tpp1)
LCO: 104921573(tpp1)
NCC: 104960361(tpp1)
TBEN: 117485661(tpp1)
CGOB: 115027224(tpp1)
PGEO: 117467392(tpp1)
GACU: 117549822(tpp1)
EMAC: 134881117(tpp1)
ELY: 117267045(tpp1)
EFO: 125901014(tpp1)
PLEP: 121953703(tpp1)
SLUC: 116035657(tpp1)
ECRA: 117936045(tpp1)
ESP: 116669910(tpp1)
PFLV: 114546617(tpp1)
GAT: 120832622(tpp1)
PPUG: 119227584(tpp1)
AFB: 129103398(tpp1)
CLUM: 117725230(tpp1)
PSWI: 130201958(tpp1)
MSAM: 119910071(tpp1)
SCHU: 122861506(tpp1)
CUD: 121526421(tpp1)
ALAT: 119004536(tpp1)
MZE: 101481977(tpp1)
ONL: 100693374(tpp1)
OAU: 116334731(tpp1)
OLA: 101174237(tpp1)
OML: 112145402(tpp1)
CSAI: 133461967(tpp1)
XMA: 102217421(tpp1)
XCO: 114134419(tpp1)
PRET: 103458893(tpp1)
PFOR: 103144264(tpp1)
PLAI: 106963704(tpp1)
PMEI: 106918270(tpp1)
GAF: 122846504(tpp1)
PPRL: 129374802(tpp1)
CVG: 107087560(tpp1)
CTUL: 119784250(tpp1)
GMU: 124855553(tpp1)
NFU: 107392300(tpp1)
KMR: 108236790(tpp1)
ALIM: 106521394(tpp1)
NWH: 119427332(tpp1)
AOCE: 111588532(tpp1)
MCEP: 125023507(tpp1)
CSEM: 103376880(tpp1)
POV: 109628108(tpp1)
SSEN: 122782426(tpp1)
HHIP: 117756363(tpp1)
HSP: 118116307(tpp1)
SMAU: 118286708(tpp1)
LCF: 108892480(tpp1) 108892632
SDU: 111225389(tpp1)
SLAL: 111644766(tpp1)
XGL: 120795311(tpp1)
HCQ: 109526111(tpp1)
SSCV: 125980400
SBIA: 133513284(tpp1)
PEE: 133412565(tpp1)
PTAO: 133487758(tpp1)
BPEC: 110155486(tpp1)
MALB: 109953961 109955168(tpp1)
BSPL: 114848170(tpp1)
SJO: 128375354(tpp1)
SASA: 100195058(tpp1)
STRU: 115162649(tpp1)
OTW: 112256401(tpp1)
OMY: 110505207(tpp1)
OGO: 124046214(tpp1)
ONE: 115138235(tpp1)
OKI: 109903816(tpp1)
OKE: 118368592(tpp1)
SALP: 112068299(tpp1)
SNH: 120023225(tpp1)
CCLU: 121545158(tpp1)
ELS: 105015977(tpp1)
SFM: 108941423(tpp1)
PKI: 111852423(tpp1)
AANG: 118226094(tpp1)
LOC: 102694809(tpp1)
PSEX: 120518532(tpp1)
LCM: 102367029(TPP1)
CMK: 103185353(tpp1)
RTP: 109928844(tpp1)
CPLA: 122553494(tpp1)
HOC: 132818353(tpp1)
LERI: 129700145(tpp1)
PMRN: 116947684(TPP1)
LRJ: 133341490(TPP1)
BFO: 118428919
BBEL: 109483414
NVE: 5513561
EPA: 110249513
ATEN: 116287345
ADF: 107356232
AMIL: 114968861
PDAM: 113678143
SPIS: 111338468
DGT: 114526386
XEN: 124446780
HMG: 100213935
HSY: 130647049
NCR: NCU08418
NTE: NEUTE1DRAFT64480(NEUTE1DRAFT_64480) NEUTE1DRAFT79855(NEUTE1DRAFT_79855)
ACHE: ACHE_40399A(SED4) ACHE_60568S(SED2) ACHE_80798S(SED1)
APUU: APUU_20891A(SED1) APUU_30508S(SED4) APUU_80775A(SED2)
ABP: AGABI1DRAFT72616(AGABI1DRAFT_72616) AGABI1DRAFT83390(AGABI1DRAFT_83390)
ABV: AGABI2DRAFT193429(AGABI2DRAFT_193429) AGABI2DRAFT195772(AGABI2DRAFT_195772)
SCM: SCHCO_02499350(SCHCODRAFT_02499350) SCHCO_02618632(SCHCODRAFT_02618632) SCHCO_02622814(SCHCODRAFT_02622814) SCHCO_02627176(SCHCODRAFT_02627176) SCHCO_02627185(SCHCODRAFT_02627185)
DDI: DDB_G0269914(tpp1)
DFA: DFA_09883 DFA_10992(tpp1)
SMIN: v1.2.016800.t1(symbB.v1.2.016800.t1) v1.2.026734.t1(symbB.v1.2.026734.t1)
 » show all
Reference
  Authors
Golabek AA, Kida E, Walus M, Wujek P, Mehta P, Wisniewski KE
  Title
Biosynthesis, glycosylation, and enzymatic processing in vivo of human tripeptidyl-peptidase I.
  Journal
J Biol Chem 278:7135-45 (2003)
DOI:10.1074/jbc.M211872200
  Sequence
[hsa:1200]
Reference
  Authors
Van Beersel G, Tihon E, Demine S, Hamer I, Jadot M, Arnould T
  Title
Different molecular mechanisms involved in spontaneous and oxidative stress-induced mitochondrial fragmentation in tripeptidyl peptidase-1 (TPP-1)-deficient fibroblasts.
  Journal
Biosci Rep 33:e00023 (2013)
DOI:10.1042/BSR20120104
  Sequence
[hsa:1200]
Reference
  Authors
Ezaki J, Takeda-Ezaki M, Oda K, Kominami E
  Title
Characterization of endopeptidase activity of tripeptidyl peptidase-I/CLN2 protein which is deficient in classical late infantile neuronal ceroid lipofuscinosis.
  Journal
Biochem Biophys Res Commun 268:904-8 (2000)
DOI:10.1006/bbrc.2000.2207
  Sequence
[rno:83534]
Reference
  Authors
Junaid MA, Wu G, Pullarkat RK
  Title
Purification and characterization of bovine brain lysosomal pepstatin-insensitive proteinase, the gene product deficient in the human late-infantile neuronal ceroid lipofuscinosis.
  Journal
J Neurochem 74:287-94 (2000)
DOI:10.1046/j.1471-4159.2000.0740287.x
  Sequence
[bta:515575]
Reference
  Authors
Reichard U, Lechenne B, Asif AR, Streit F, Grouzmann E, Jousson O, Monod M
  Title
Sedolisins, a new class of secreted proteases from Aspergillus fumigatus with endoprotease or tripeptidyl-peptidase activity at acidic pHs.
  Journal
Appl Environ Microbiol 72:1739-48 (2006)
DOI:10.1128/AEM.72.3.1739-1748.2006
  Sequence
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