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Functional differences in the catabolism of branched-chain l-amino acids in cultured normal and maple syrup urine disease fibroblasts

✍ Scribed by Peter Schadewaldt; Udo Wendel

Book ID
113382763
Publisher
Elsevier Science
Year
1989
Tongue
English
Weight
825 KB
Volume
41
Category
Article
ISSN
0885-4505

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## Abstract Individuals with maple syrup urine disease (MSUD) have an inherited metabolic disorder resulting in a deficiency in the branched‐chain keto‐acid dehydrogenase complex. As a result, these individuals have elevated concentrations of the branched‐chain amino acids valine, isoluecine, allo‐