Enzymology of the branched‐chain amino acid oxidation disorders: the valine pathway

Journal of Inherited Metabolic Disease - Tập 35 Số 1 - Trang 5-12 - 2012
Ronald J. A. Wanders1, Marinus Durán1, Ference J. Loupatty2,1
1Head Lab Genetic Metabolic Diseases, Room F0‐226 Academic Medical Center University of Amsterdam Meibergdreef 9 Amsterdam 1105 AZ The Netherlands
2Department of Clinical Chemistry Onze Lieve Vrouwe Gasthuis (OLVG) Amsterdam The Netherlands

Tóm tắt

AbstractValine is one of the three branched‐chain amino acids which undergoes oxidation within mitochondria. In this paper, we describe the current state of knowledge with respect to the enzymology of the valine oxidation pathway and the different disorders affecting oxidation.

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