Article
Structure of tripeptidyl-peptidase I provides insight into the molecular basis of late infantile neuronal ceroid lipofuscinosis.
The Journal of biological chemistry - 6 Feb 2009
Pal Aritra, Kraetzner Ralph, Gruene Tim, Grapp Marcel, Schreiber Kathrin, Grønborg Mads, Urlaub Henning, Becker Stefan, Asif Abdul R, Gärtner Jutta, Sheldrick George M, Steinfeld Robert
Abstract excerpt
Late infantile neuronal ceroid lipofuscinosis, a fatal neurodegenerative disease of childhood, is caused by mutations in the TPP1 gene that encodes tripeptidyl-peptidase I. We show that purified TPP1 requires at least partial glycosylation for in vitro autoprocessing and proteolytic activity. We crystallized the fully glycosylated TPP1 precursor under conditions that implied partial autocatalytic cleavage between...
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