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Inhibition of iduronic acid biosynthesis by ebselen reduces glycosaminoglycan accumulation in mucopolysaccharidosis type I fibroblasts

Mucopolysaccharidosis type I (MPS-I) is a rare lysosomal storage disorder caused by deficiency of the enzyme alpha-L-iduronidase, which removes iduronic acid in both chondroitin/dermatan sulfate (CS/DS) and heparan sulfate (HS) and thereby contributes to the catabolism of glycosaminoglycans (GAGs)....

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Detalles Bibliográficos
Autores principales:	Maccarana, Marco, Tykesson, Emil, Pera, Edgar M, Gouignard, Nadège, Fang, Jianping, Malmström, Anders, Ghiselli, Giancarlo, Li, Jin-ping
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2021
Materias:	Genetic Disorders of Glycosylation
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8600295/ https://www.ncbi.nlm.nih.gov/pubmed/34192316 http://dx.doi.org/10.1093/glycob/cwab066

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