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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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| Publicado no: | Biochim Biophys Acta |
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| Main Authors: | , , , , , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Elsevier Pub. Co
2018
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC6134197/ https://ncbi.nlm.nih.gov/pubmed/30076962 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.bbadis.2018.07.031 |
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