A Critical Tryptophan and Ca(2+) in Activation and Catalysis of TPPI, the Enzyme Deficient in Classic Late-Infantile Neuronal Ceroid Lipofuscinosis

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 s...

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Главные авторы: 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.
Формат: Artigo
Язык:Inglês
Опубликовано: Public Library of Science 2010
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Research Article
Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC2914745/
https://ncbi.nlm.nih.gov/pubmed/20689811
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1371/journal.pone.0011929
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