Rabbit Monoclonal Antibody to TPP1

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, Jul 2008]