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Four novel mutations identified in Norwegian patients result in intermittent maple syrup urine disease when combined with the R301C mutation
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Oxidative stress in plasma from maple syrup urine disease patients during treatment
Production and characterization of murine models of classic and intermediate maple syrup urine disease
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Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain α-keto acids accumulating in maple syrup urine disease
Biochemical correlates of neuropsychiatric illness in maple syrup urine disease
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Inhibition of brain energy metabolism by the α-keto acids accumulating in maple syrup urine disease
Evidence that oxidative stress is increased in plasma from patients with maple syrup urine disease
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Evidence that the branched-chain alpha-keto acids accumulating in maple syrup urine disease induce morphological alterations and death in cultured astrocytes from rat cerebral cortex
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