Glucose-free/high-protein diet improves hepatomegaly and exercise intolerance in glycogen storage disease type III mice

Glycogen disease type III (GSDIII), a rare incurable autosomal recessive disorder due to glycogen debranching enzyme deficiency, presents with liver, heart and skeletal muscle impairment, hepatomegaly and ketotic hypoglycemia. Muscle weakness usually worsens to fixed myopathy and cardiac involvement...

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Detalles Bibliográficos
Autores principales: Pagliarani, Serena, Lucchiari, Sabrina, Ulzi, Gianna, Ripolone, Michela, Violano, Raffaella, Fortunato, Francesco, Bordoni, Andreina, Corti, Stefania, Moggio, Maurizio, Bresolin, Nereo, Comi, Giacomo P.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier Pub. Co 2018
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6134197/
https://www.ncbi.nlm.nih.gov/pubmed/30076962
http://dx.doi.org/10.1016/j.bbadis.2018.07.031

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