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.

单词	tpp1
释义	TPP1 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.
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