Regulation of the branched-chain α-ketoacid dehydrogenase and elucidation of a molecular basis for maple syrup urine disease
RA Harris, B Zhang, GW Goodwin, MJ Kuntz… - Advances in Enzyme …, 1990 - Elsevier
The hepatic branched-chain α-ketoacid dehydrogenase complex plays an important role in
regulating branched-chain amino acid levels. These compounds are essential for protein
synthesis but toxic if present in excess. When dietary protein is deficient, the hepatic enzyme
is converted to the inactive, phosphorylated state to conserve branched-chain amino acids
for protein synthesis. When dietary protein is excessive, the enzyme is in the active,
dephosphorylated state to commit the excess branched-chain amino acids to degradation …
regulating branched-chain amino acid levels. These compounds are essential for protein
synthesis but toxic if present in excess. When dietary protein is deficient, the hepatic enzyme
is converted to the inactive, phosphorylated state to conserve branched-chain amino acids
for protein synthesis. When dietary protein is excessive, the enzyme is in the active,
dephosphorylated state to commit the excess branched-chain amino acids to degradation …