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Classical maple syrup urine disease and brain development: Principles of management and formula design

Branched-chain ketoacid dehydrogenase deficiency results in complex and volatile metabolic derangements that threaten brain development. Treatment for classical maple syrup urine disease (MSUD) should address this underlying physiology while also protecting children from nutrient deficiencies. Based...

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Hlavní autoři: Strauss, Kevin A., Wardley, Bridget, Robinson, Donna, Hendrickson, Christine, Rider, Nicholas L., Puffenberger, Erik G., Shelmer, Diana, Moser, Ann B., Morton, D. Holmes
Médium: Artigo
Jazyk:Inglês
Vydáno: 2010
Témata:
On-line přístup:https://ncbi.nlm.nih.gov/pmc/articles/PMC3671925/
https://ncbi.nlm.nih.gov/pubmed/20061171
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2009.12.007
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