TPP1

(redirected from TPP-1)

TPP1

A gene on chromosome 11p15 that encodes a sedolisin-type serine protease, which acts in lysosomes to cleave N-terminal tripeptides from substrates; it also has weak endopeptidase activity. Tripeptidyl peptidase I is activated and auto-proteolysed by acidification.

Molecular pathology
TPP1 mutations have been linked to late-infantile neuronal ceroid lipofuscinosis (CLN2, Jansky-Bielschowsky disease), which is due to the failure to degrade specific neuropeptides, and a subunit of ATP synthase in lysosomes.
Mentioned in ?
References in periodicals archive ?
New power units fitted with similar equipment, which was also manufactured and supplied by Power Machines, were put into service at Permskaya TPP-9 (TGC-9 OJSC), Izhevskaya TPP-1 (TGC-5 OJSC) and Vladimirskaya TPP-2 (TGC-6 OJSC) this year.
Kaluga Turbine Works OJSC member of the Power Machines Company has concluded a contract to supply power generation equipment for Voronezhskaya TPP-1.