Article
A critical tryptophan and Ca2+ in activation and catalysis of TPPI, the enzyme deficient in classic late-infantile neuronal ceroid lipofuscinosis.
PloS one - 3 Aug 2010
Kuizon Salomon, DiMaiuta Kathleen, Walus Marius, Jenkins Edmund C, Kuizon Marisol, Kida Elizabeth, Golabek Adam A, Espinoza Daniel O, Pullarkat Raju K, Junaid Mohammed A
Abstract excerpt
BACKGROUND: Tripeptidyl aminopeptidase I (TPPI) is a crucial lysosomal enzyme that is deficient in the fatal neurodegenerative disorder called classic late-infantile neuronal ceroid lipofuscinosis (LINCL). It is involved in the catabolism of proteins in the lysosomes. Recent X-ray crystallographic studies have provided insights into the structural/functional aspects of TPPI catalysis, and indicated presence of an...
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