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Gene silencing of EXTL2 and EXTL3 as a substrate deprivation therapy for heparan sulphate storing mucopolysaccharidoses

Neurological pathology is characteristic of the mucopolysaccharidoses (MPSs) that store heparan sulphate (HS) glycosaminoglycan (gag) and has been proven to be refractory to systemic therapies. Substrate deprivation therapy (SDT) using general inhibitors of gag synthesis improves neurological functi...

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Detalhes bibliográficos
Main Authors: Kaidonis, Xenia, Liaw, Wan Chin, Roberts, Ainslie Derrick, Ly, Marleesa, Anson, Donald, Byers, Sharon
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group 2010
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2987189/
https://ncbi.nlm.nih.gov/pubmed/19690583
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/ejhg.2009.143
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