Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease
โ Scribed by Y. Shigematsu; K. Kikuchi; T. Momoi; M. Sudo; Y. Kikawa; K. Nosaka; M. Kuriyama; S. Haruki; K. Sanada; N. Hamano; Y. Suzuki
- Publisher
- Springer
- Year
- 1983
- Tongue
- English
- Weight
- 849 KB
- Volume
- 6
- Category
- Article
- ISSN
- 0141-8955
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๐ SIMILAR VOLUMES
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
## Abstract Individuals with maple syrup urine disease (MSUD) have an inherited metabolic disorder resulting in a deficiency in the branchedโchain ketoโacid dehydrogenase complex. As a result, these individuals have elevated concentrations of the branchedโchain amino acids valine, isoluecine, alloโ
## 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