Immunoprecipitation of TPP1 transfected lysate using anti-TPP1 MaxPab rabbit polyclonal antibody and Protein A Magnetic Bead (U0007), and immunoblotted with TPP1 purified MaxPab mouse polyclonal antibody (B01P) (H00001200-B01P).
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
ceroid-lipofuscinosis, neuronal 2, late infantile (Jansky-Bielschowsky disease),growth-inhibiting protein 1,lysosomal pepstatin insensitive protease,tripeptidyl aminopeptidase,tripeptidyl-peptidase I