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Description
This gene encodes 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. [provided by RefSeq
Specifications
Specifications
| Antigen | TPP1 |
| Applications | ELISA, Immunohistochemistry (PFA fixed), Western Blot |
| Classification | Monoclonal |
| Clone | 3B1 |
| Conjugate | Unconjugated |
| Description | Mouse monoclonal antibody raised against a partial recombinant TPP1. |
| Formulation | PBS with no preservative; pH 7.4 |
| Gene | TPP1 |
| Gene Accession No. | BC014863 |
| Gene Alias | CLN2/GIG1/LPIC/MGC21297 |
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