| Entry |
|
| Name |
tripeptidyl-peptidase I;
tripeptidyl aminopeptidase;
tripeptidyl peptidase
|
| Class |
Hydrolases;
Acting on peptide bonds (peptidases);
Dipeptidyl-peptidases and tripeptidyl-peptidases
 |
| 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 |
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| Genes |
HSA: | | PTR: | | PPS: | | GGO: | | PON: | | MCC: | | MMU: | | RNO: | | CFA: | | AML: | | FCA: | | BTA: | | SSC: | | ECB: | | MDO: | | SHR: | | OAA: | | GGA: | | TGU: | | ACS: | | XLA: | | XTR: | | DRE: | | TRU: | | OLA: | | BFO: | | NVE: | | HMG: | | AQU: | | NCR: | | SMP: | | PAN: | | TTT: | | MTM: | | MGR: | | FGR: | | NHE: | | VAL: | | SSL: | | BFU: | | ANI: | | NFI: | | AFM: | | AOR: | | ANG: | | 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
 |
| Reference |
|
| 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 |
|
| Reference |
|
| 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 |
|
| 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 |
|
| 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 |
|
| Reference |
|
| 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 |
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| Other DBs |
ExplorEnz - The Enzyme Database: IUBMB Enzyme Nomenclature: ExPASy - ENZYME nomenclature database: BRENDA, the Enzyme Database: CAS: 151662-36-1 |