Effects of flavonoids on glycosaminoglycan synthesis: implications for substrate reduction therapy in Sanfilippo disease and other mucopolysaccharidoses

Sanfilippo disease (mucopolysaccharidosis type III, MPS III) is a severe metabolic disorder caused by accumulation of heparan sulfate (HS), one of glycosaminoglycans (GAGs), due to a genetic defect resulting in a deficiency of GAG hydrolysis. This disorder is characterized as the most severe neurolo...

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Autori principali: Kloska, Anna, Jakóbkiewicz-Banecka, Joanna, Narajczyk, Magdalena, Banecka-Majkutewicz, Zyta, Węgrzyn, Grzegorz
Natura: Artigo
Lingua:Inglês
Pubblicazione: Springer US 2011
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Original Paper
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC3070076/
https://ncbi.nlm.nih.gov/pubmed/21305347
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s11011-011-9233-2
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