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3-hydroxyisobutyryl-coa hydroxyisobutyryl coa valine dehydrogenase oxidation pathway hydroxyisobutyric isobutyryl hydrolase deficiency branched chain amino acid enoyl hydratase biochemical characterization hibch acetyl methylmalonyl fatty propionyl basal ganglia mitochondrial deacylase protein 3d rendering chemical molecule molecular structural structure formula substance metabolite metabolism biosynthesis path catabolism biosynthetic hmdb intermediate precursor synthesis derivative