IVD is a mitochondrial flavoprotein, a tetramer of 43 kDa subunits, which catalyzes the third reaction in leucine metabolism. Hereditary deficiency of IVD causes isovaleric acidemia, one of the ...

In this study we evaluate a 9 year old female whose cultured fibroblasts have 4% of control isovaleryl-CoA dehydrogenase activity. Glycine supplements between 0 and 600 mg/kg/d were compared at ...