Morphological alterations and induction of oxidative stress in glial cells caused by the branched-chain α-keto acids accumulating in maple syrup urine disease
C Funchal, A Latini, MC Jacques-Silva… - Neurochemistry …, 2006 - Elsevier
Maple syrup urine disease (MSUD) is an inherited neurometabolic disorder biochemically
characterized by the accumulation of the branched-chain α-keto acids (BCKA) α-
ketoisocaproic (KIC), α-keto-β-methylvaleric (KMV) and α-ketoisovaleric (KIV) and their
respective branched-chain α-amino acids in body fluids and tissues. Affected MSUD patients
have predominantly neurological features, including cerebral edema and atrophy whose
pathophysiology is not well established. In the present study we investigated the effects of …
characterized by the accumulation of the branched-chain α-keto acids (BCKA) α-
ketoisocaproic (KIC), α-keto-β-methylvaleric (KMV) and α-ketoisovaleric (KIV) and their
respective branched-chain α-amino acids in body fluids and tissues. Affected MSUD patients
have predominantly neurological features, including cerebral edema and atrophy whose
pathophysiology is not well established. In the present study we investigated the effects of …