Tripeptidyl Peptidase I (TPP1)

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CLN2; GIG1; LPIC; Tripeptidyl aminopeptidase; Lysosomal pepstatin-insensitive protease; Ceroid-Lipofuscinosis,Neuronal 2,Late Infantile; Cell growth-inhibiting gene 1 protein

Tripeptidyl Peptidase I (TPP1)
Tripeptidyl-peptidase 1 is a member of the sedolisin family of serine proteases. The protease functions in the lysosome to cleave N-terminal tripeptides from substrates, and has weaker endopeptidase activity.
It is synthesized as a catalytically-inactive enzyme which is activated and auto-proteolyzed upon acidification. Mutations in this gene result in late-infantile neuronal ceroid lipofuscinosis, which is associated with the failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome.Detected in all tissues examined with highest levels in heart and placenta and relatively similar levels in other tissues.

Organism species: Homo sapiens (Human)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
ProteinsRPC828Hu01Recombinant Tripeptidyl Peptidase I (TPP1)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAC828Hu01Polyclonal Antibody to Tripeptidyl Peptidase I (TPP1)WB, ICC, IHC-P, IHC-F, ELISA
MAC828Hu22Monoclonal Antibody to Tripeptidyl Peptidase I (TPP1)WB, ICC, IHC-P, IHC-F, ELISA
Assay KitsSEC828HuELISA Kit for Tripeptidyl Peptidase I (TPP1)Enzyme-linked immunosorbent assay

Organism species: Mus musculus (Mouse)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
ProteinsRPC828Mu01Recombinant Tripeptidyl Peptidase I (TPP1)SDS-PAGE; WB; ELISA; IP.
RPC828Mu02Recombinant Tripeptidyl Peptidase I (TPP1)SDS-PAGE; WB; ELISA; IP.
AntibodiesPAC828Mu01Polyclonal Antibody to Tripeptidyl Peptidase I (TPP1)WB, ICC, IHC-P, IHC-F, ELISA
PAC828Mu02Polyclonal Antibody to Tripeptidyl Peptidase I (TPP1)WB, ICC, IHC-P, IHC-F, ELISA
Assay KitsSEC828MuELISA Kit for Tripeptidyl Peptidase I (TPP1)Enzyme-linked immunosorbent assay

Organism species: Rattus norvegicus (Rat)

CATALOG NO.PRODUCT NAMEAPPLICATIONS
Proteinsn/aRecombinant Tripeptidyl Peptidase I (TPP1)Recombinant Protein Customized Service Offer
Antibodiesn/aMonoclonal Antibody to Tripeptidyl Peptidase I (TPP1)Monoclonal Antibody Customized Service Offer
n/aPolyclonal Antibody to Tripeptidyl Peptidase I (TPP1)Polyclonal Antibody Customized Service Offer
Assay Kitsn/aCLIA Kit for Tripeptidyl Peptidase I (TPP1)CLIA Kit Customized Service Offer
n/aELISA Kit for Tripeptidyl Peptidase I (TPP1)ELISA Kit Customized Service Offer
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  20. "Expression and analysis of CLN2 variants in CHO cells: Q100R represents a polymorphism, and G389E and R447H represent loss-of-function mutations."Hum. Mutat. 18:165-165(2001) [PubMed] [Europe PMC] [Abstract]
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  24. "Mutation of the glycosylated asparagine residue 286 in human CLN2 protein results in loss of enzymatic activity."Glycobiology 14:1C-5C(2004) [PubMed] [Europe PMC] [Abstract]
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  26. "Functional consequences and rescue potential of pathogenic missense mutations in tripeptidyl peptidase I."Hum. Mutat. 31:710-721(2010) [PubMed] [Europe PMC] [Abstract]
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