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Branched-chain alpha-keto acids isolated as oxime derivatives: Relationship to the corresponding hydroxy acids and amino acids in Maple Syrup Urine Disease

✍ Scribed by Gerald Lancaster; Orval A. Mamer; Charles R. Scriver

Book ID: 118947366
Publisher: Elsevier Science
Year: 1974
Tongue: English
Weight: 703 KB
Volume: 23
Category: Article
ISSN: 1532-8600
DOI: 10.1016/0026-0495(74)90064-x

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## Abstract Severe neurological symptoms, cerebral edema, and atrophy are common features of the inherited metabolic disorder maple syrup urine disease (MSUD). However, the pathomechanisms involved in the neuropathology of this disease are not well established. In this study, we investigated the ef