Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
Volume 1639, Issue 3, 20 November 2003, Pages 232-238
Inhibition of brain energy metabolism by the α-keto acids accumulating in maple syrup urine disease
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Keywords
Maple syrup urine disease
α-Ketoisocaproic acid
α-Keto-β-methylvaleric acid
α-Ketoisovaleric acid
Energy metabolism
Abbreviations
MSUD
maple syrup urine disease
BCAA
branched chain amino acids
BCKA
branched chain keto acids
BCKAD
branched chain l-α-keto acid dehydrogenase
KIC
α-ketoisocaproic acid
KMV
α-keto-β-methylvaleric acid
KIV
α-ketoisovaleric acid
CNS
central nervous system
GABA
gamma-aminobutyric acid
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