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Energy Expenditure in Chilean Children with Maple Syrup Urine Disease (MSUD)
Introduction: Maple syrup urine disease (MSUD) is an autosomal recessive disorder caused by a blockage of branched-chain keto acid of BCAA (branched-chain keto acid dehydrogenase, BCKDH) leading to neurological damage induced by accumulation of leucine and metabolites. MSUD expenditure and energy re...
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| Publicado no: | JIMD Rep |
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| Main Authors: | , , , , , , |
| Formato: | Artigo |
| Idioma: | Inglês |
| Publicado em: |
Springer Berlin Heidelberg
2015
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| Assuntos: | |
| Acesso em linha: | https://ncbi.nlm.nih.gov/pmc/articles/PMC4864776/ https://ncbi.nlm.nih.gov/pubmed/26458887 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/8904_2015_500 |
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