Enzymatic analysis of individual posterior root ganglion cells in olivopontocerebellar atrophy, amyotrophic lateral sclerosis and Duchenne muscular dystrophy.
Four enzyme activities related to glucose metabolism, i.e. those of glucose-6-phosphate dehydrogenase (G6PDH; EC 1.1.1.49), lactic dehydrogenase (LDH; EC 1.1.1.27), pyruvate dehydrogenase complex (PDC) and citrate synthase (CS; EC 4.1.3.7) were estimated in posterior root ganglion cells (PRGCs) of the spinal cord in patients suffering from olivopontocerebellar atrophy (OPCA), amyotrophic lateral sclerosis (ALS), and Duchenne muscular dystrophy (DMD) by means of the NAD, NADP and CoA cycling methods. In ALS and DMD, the enzyme activities examined were within normal ranges. In OPCA, PDC activity was significantly reduced and LDH activity tended to be lower than that in controls.
D000293:Adolescent; D000328:Adult; D000368:Aged; D000690:Amyotrophic Lateral Sclerosis / Q000201:enzymology*; D002524:Cerebellar Ataxia / Q000201:enzymology*; D002950:Citrate (si)-Synthase / Q000378:metabolism; D005260:Female; D005727:Ganglia, Spinal / Q000201:enzymology*; D005954:Glucosephosphate Dehydrogenase / Q000378:metabolism; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D008875:Middle Aged; D009136:Muscular Dystrophies / Q000201:enzymology*; D009847:Olivary Nucleus; D011149:Pons; D011768:Pyruvate Dehydrogenase Complex / Q000378:metabolism; D013577:Syndrome
D011768:Pyruvate Dehydrogenase Complex; D007770:L-Lactate Dehydrogenase; D005954:Glucosephosphate Dehydrogenase; D002950:Citrate (si)-Synthase