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Branched-chain L-amino acid metabolism in classical maple syrup urine disease after orthotopic liver transplantation

✍ Scribed by A. Bodner-Leidecker; U. Wendel; J-M. Saudubray; P. Schadewaldt

Book ID
111567653
Publisher
Springer
Year
2000
Tongue
English
Weight
208 KB
Volume
23
Category
Article
ISSN
0141-8955

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✍ 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