tripeptidyl-peptidase 1

tripeptidyl-peptidase 1

An enzyme encoded by TPP1 on chromosome 11p15, which belongs to the sedolisin family of serine proteases and cleaves N-terminal tripeptides from substrates in lysosomes.

Molecular pathology
TPP1 mutations result in a failure to degrade specific neuropeptides and a subunit of ATP synthase in the lysosome, resulting in late infantile neuronal ceroid lipofuscinosis (Jansky-Bielschowsky disease).
References in periodicals archive ?
24,25) Assays for palmitoyl-protein thioesterase 1 and tripeptidyl-peptidase 1 are clinically available.