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Neural deficits contribute to respiratory insufficiency in Pompe disease

Pompe disease is a severe form of muscular dystrophy due to glycogen accumulation in all tissues, especially striated muscle. Disease severity is directly related to the deficiency of acid α-glucosidase (GAA), which degrades glycogen in the lysosome. Respiratory dysfunction is a hallmark of the dise...

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Detalhes bibliográficos
Main Authors: DeRuisseau, Lara R., Fuller, David D., Qiu, Kai, DeRuisseau, Keith C., Donnelly, William H., Mah, Cathryn, Reier, Paul J., Byrne, Barry J.
Formato: Artigo
Idioma:Inglês
Publicado em: National Academy of Sciences 2009
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2695054/
https://ncbi.nlm.nih.gov/pubmed/19474295
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.0902534106
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