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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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Detalles Bibliográficos
Autores principales: 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.
Formato: Texto
Lenguaje:English
Publicado: Public Library of Science 2010
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2914745/
https://www.ncbi.nlm.nih.gov/pubmed/20689811
http://dx.doi.org/10.1371/journal.pone.0011929