Inhibition of brain energy metabolism by the α-keto acids accumulating in maple syrup urine disease
- 20 November 2003
- journal article
- Published by Elsevier in Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
- Vol. 1639 (3) , 232-238
- https://doi.org/10.1016/j.bbadis.2003.09.010
Abstract
No abstract availableKeywords
This publication has 38 references indexed in Scilit:
- α-Ketoisocaproate Alters the Production of Both Lactate and Aspartate from [U-13C]Glutamate in Astrocytes: A 13C NMR StudyJournal of Neurochemistry, 2002
- Chronic early leucine administration induces behavioral deficits in ratsLife Sciences, 1999
- An Evaluation of the Measurement of the Activities of Complexes I-IV in the Respiratory Chain of Human Skeletal Muscle MitochondriaBiochemical Medicine and Metabolic Biology, 1994
- Irreversible Inhibition of Mitochondrial Complex I by 1‐Methyl‐4‐Phenylpyridinium: Evidence for Free Radical InvolvementJournal of Neurochemistry, 1992
- Maple syrup urine disease: Interrelations between branched‐chain amino‐, oxo‐ and hydroxyacids; implications for treatment; associations with CNS dysmyelinationJournal of Inherited Metabolic Disease, 1991
- Effects of methylmalonate and propionate on uptake of glucose and ketone bodies in vitro by brain of developing ratsBiochemical Medicine and Metabolic Biology, 1991
- Anatomic and Disease Specificity of NADH CoQ1 Reductase (Complex I) Deficiency in Parkinson's DiseaseJournal of Neurochemistry, 1990
- Myelin proteins: Degradation in rat brain initiated by metabolites causative of maple syrup urine diseaseBiochemical and Biophysical Research Communications, 1983
- Dopamine‐β‐hydroxylase activity in human cerebrospinal fluidJournal of Neurochemistry, 1976
- Determination of blood glucose using an oxidase-peroxidase system with a non-carcinogenic chromogenJournal of Clinical Pathology, 1969