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Elevated excretion of N-acetylated branched-chain amino acids in maple syrup urine disease

✍ Scribed by W. Lehnert; E. Werle


Book ID
115826097
Publisher
Elsevier Science
Year
1988
Tongue
English
Weight
256 KB
Volume
172
Category
Article
ISSN
0009-8981

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Maple syrup urine disease: Branched-chai
✍ Stephen D. Skaper; Daniel P. Molden; J. Edwin Seegmiller πŸ“‚ Article πŸ“… 1976 πŸ› Springer 🌐 English βš– 707 KB

The intracellular concentration of free leucine, isoleucine, and valine and their metabolism were studied in lymphoblast cultures established from peripheral blood of an individual with maple syrup urine disease (MSUD) and a control subject. Branched-chain alpha-keto acid decarboxylase activity in t