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...

Disgrifiad llawn

Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Prif Awduron: Strauss, Kevin A., Wardley, Bridget, Robinson, Donna, Hendrickson, Christine, Rider, Nicholas L., Puffenberger, Erik G., Shelmer, Diana, Moser, Ann B., Morton, D. Holmes
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: 2010
Pynciau:
Article
Mynediad Ar-lein: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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