Lysosomal enzyme phosphorylation in human fibroblasts. Kinetic parameters offer a biochemical rationale for two distinct defects in the uridine diphospho-N-acetylglucosamine:lysosomal enzyme precursor N-acetylglucosamine-1-phosphotransferase.

The primary genetic defect in the lysosomal storage disease mucolipidosis III (ML III) is in the enzyme uridine diphospho-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferase. This enzyme has two well-defined functions: specific recognition of lysosomal enzymes (recognition...

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Detalhes bibliográficos
Publicado no:J Clin Invest
Main Authors: Lang, L, Takahashi, T, Tang, J, Kornfeld, S
Formato: Artigo
Idioma:Inglês
Publicado em: American Society for Clinical Investigation 1985
Assuntos:
Research Article
Acesso em linha:https://ncbi.nlm.nih.govhttps://pmc.ncbi.nlm.nih.gov/articles/PMC424341/
https://ncbi.nlm.nih.govhttps://pubmed.ncbi.nlm.nih.gov/3001146/
https://ncbi.nlm.nih.govhttps://doi.org/10.1172/JCI112227
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