Cytoskeleton as a potential target in the neuropathology of maple syrup urine disease: Insight from animal studies

Abstract

In this short review we provide evidence that the branched-chain keto acids accumulating in the neurometabolic disorder maple syrup urine disease disturb rat cerebral cytoskeleton in a developmentally regulated manner. Alterations of protein phosphorylation leading to brain cytoskeletal misregulation and neural cell death caused by these metabolites are… (More)
DOI: 10.1007/s10545-007-0562-6

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