Variation in the levels of complex I subunits among tissues in a patient with mitochondrial encephalomyopathy and renal dysfunction.
Enzymic activity and the levels of immunochemically detectable subunits of NADH-ubiquinone oxidoreductase (Complex I) were measured in the mitochondria from various tissues of a patient with mitochondrial encephalomyopathy and renal dysfunction. Rotenone-sensitive NADH-cytochrome c reductase activity was decreased in all the tissues examined, but the degree of deficiency varied from tissue to tissue. The levels of subunits in Complex I were decreased roughly in parallel with the activity in each tissue. These results indicate that the apparently tissue-specific manifestation of symptoms depends mainly on the levels of subunits in Complex I.
D000293:Adolescent; D001927:Brain Diseases / Q000150:complications / Q000201:enzymology*; D006801:Humans; D007668:Kidney / Q000201:enzymology; D007676:Kidney Failure, Chronic / Q000150:complications / Q000201:enzymology*; D008297:Male; D008928:Mitochondria / Q000201:enzymology*; D008929:Mitochondria, Heart / Q000201:enzymology; D008930:Mitochondria, Liver / Q000201:enzymology; D009132:Muscles / Q000201:enzymology; D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases / Q000302:isolation & purification*
D016660:NAD(P)H Dehydrogenase (Quinone); D011808:Quinone Reductases