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Structural and mechanistic insight into the basis of mucopolysaccharidosis IIIB

Mucopolysaccharidosis III (MPS III) has four forms (A–D) that result from buildup of an improperly degraded glycosaminoglycan in lysosomes. MPS IIIB is attributable to the decreased activity of a lysosomal α-N-acetylglucosaminidase (NAGLU). Here, we describe the structure, catalytic mechanism, and i...

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Detalhes bibliográficos
Main Authors: Ficko-Blean, Elizabeth, Stubbs, Keith A., Nemirovsky, Oksana, Vocadlo, David J., Boraston, Alisdair B.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2008
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2373330/
https://ncbi.nlm.nih.gov/pubmed/18443291
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0711491105
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