Biochemical properties of mammalian 2-oxo acid dehydrogenase multienzyme complexes and clinical relevancy with chronic lactic acidosis.
D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide); D000138:Acidosis / Q000151:congenital* / Q000201:enzymology; D000818:Animals; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007655:Ketoglutarate Dehydrogenase Complex / Q000378:metabolism; D007658:Ketone Oxidoreductases / Q000037:antagonists & inhibitors / Q000378:metabolism*; D007700:Kinetics; D007773:Lactates / Q000378:metabolism*; D019344:Lactic Acid; D008099:Liver / Q000201:enzymology; D046911:Macromolecular Substances; D008297:Male; D008854:Microscopy, Electron; D008929:Mitochondria, Heart / Q000201:enzymology*; D009097:Multienzyme Complexes / Q000037:antagonists & inhibitors / Q000378:metabolism*; D011768:Pyruvate Dehydrogenase Complex / Q000378:metabolism; D011773:Pyruvates / Q000378:metabolism*; D019289:Pyruvic Acid; D013329:Structure-Activity Relationship; D013552:Swine
D007773:Lactates; D046911:Macromolecular Substances; D009097:Multienzyme Complexes; D011768:Pyruvate Dehydrogenase Complex; D011773:Pyruvates; D019344:Lactic Acid; D019289:Pyruvic Acid; D007658:Ketone Oxidoreductases; D007655:Ketoglutarate Dehydrogenase Complex; D042942:3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide)