KEGG   ENZYME: 3.4.14.9Help
Entry
EC 3.4.14.9                 Enzyme                                 

Name
tripeptidyl-peptidase I;
tripeptidyl aminopeptidase;
tripeptidyl peptidase
Class
Hydrolases;
Acting on peptide bonds (peptidases);
Dipeptidyl-peptidases and tripeptidyl-peptidases
BRITE hierarchy
Reaction(IUBMB)
Release of an N-terminal tripeptide from a polypeptide, but also has endopeptidase activity.
Comment
A lysosomal enzyme that is active at acidic pH. Deficient in classical late-infantile neuronal ceroid lipofuscinosis brain tissue. Belongs in peptidase family S53. Formerly included in EC 3.4.14.8.
Orthology
K01279  
tripeptidyl-peptidase I
Genes
HSA: 
1200(TPP1)
PTR: 
450999(TPP1)
PPS: 
100969179(TPP1)
GGO: 
101149790(TPP1)
PON: 
100440001(TPP1)
MCC: 
MMU: 
12751(Tpp1)
RNO: 
83534(Tpp1)
CFA: 
485337(TPP1)
AML: 
FCA: 
101093296(TPP1)
BTA: 
515575(TPP1)
SSC: 
ECB: 
MDO: 
SHR: 
100918462(TPP1)
OAA: 
GGA: 
425576(TPP1)
TGU: 
100232321(TPP1)
ACS: 
XLA: 
414505(tpp1)
XTR: 
100124868(tpp1)
DRE: 
798347(tpp1)
TRU: 
OLA: 
BFO: 
NVE: 
HMG: 
AQU: 
NCR: 
SMP: 
PAN: 
TTT: 
MTM: 
MGR: 
FGR: 
NHE: 
VAL: 
SSL: 
BFU: 
ANI: 
NFI: 
AFM: 
AOR: 
AOR_1_122124(AO090166000084) AOR_1_386054(AO090011000235)
ANG: 
ANI_1_368124(An14g02470) ANI_1_676074(An08g04640)
AFV: 
ACT: 
PCS: 
CIM: 
CPW: 
PBL: 
URE: 
ABE: 
TVE: 
AJE: 
PNO: 
PTE: 
ZTR: 
PPL: 
LBC: 
MPR: 
CCI: 
SCM: 
UMA: 
PGR: 
MBR: 
NGR: 
DDI: 
DPP: 
PIF: 
 » show all
Taxonomy
Reference
1  [PMID:10349869]
  Authors
Ezaki J, Tanida I, Kanehagi N, Kominami E.
  Title
A lysosomal proteinase, the late infantile neuronal ceroid lipofuscinosis gene (CLN2) product, is essential for degradation of a hydrophobic protein, the subunit c of ATP synthase.
  Journal
J. Neurochem. 72 (1999) 2573-82.
  Organism
Homo sapiens [GN:hsa]
Reference
2  [PMID:9989235]
  Authors
Rawlings ND, Barrett AJ.
  Title
Tripeptidyl-peptidase I is apparently the CLN2 protein absent in classical late-infantile neuronal ceroid lipofuscinosis.
  Journal
Biochim. Biophys. Acta. 1429 (1999) 496-500.
  Organism
Homo sapiens [GN:hsa], Rattus norvegicus [GN:rno]
Reference
3  [PMID:10679303]
  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 (2000) 904-8.
  Organism
Rattus norvegicus [GN:rno]
Reference
4  [PMID:10617131]
  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 (2000) 287-94.
  Organism
Bos taurus [GN:bta]
Reference
5  [PMID:11054422]
  Authors
Lin L, Sohar I, Lackland H, Lobel P.
  Title
The human CLN2 protein/tripeptidyl-peptidase I is a serine protease that autoactivates at acidic pH.
  Journal
J. Biol. Chem. 276 (2001) 2249-55.
  Organism
Homo sapiens [GN:hsa]
Other DBs
ExplorEnz - The Enzyme Database: 
IUBMB Enzyme Nomenclature: 
ExPASy - ENZYME nomenclature database: 
BRENDA, the Enzyme Database: 
CAS: 
151662-36-1

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