Normal rhodanese activity in leukocytes from Leber patients: enzyme characterization and activity levels.
The thiosulfate:cyanide sulfurtransferase (rhodanese) enzyme (EC 2.8.1.1.) was studied in human leukocytes from control subjects and from nine patients with Leber's hereditary optic atrophy. Enzyme activity was proportional to protein concentration in the tested range (0.09 to 0.39 mg) as well as to incubation time. The optimal pH for reaction was 8.7 and the apparent Km for thiosulfate was 7 X 10(-3) M. No significant difference of enzyme activity was present in Leber's disease.
D005260:Female; D030342:Genetic Diseases, Inborn / Q000201:enzymology*; D006801:Humans; D007962:Leukocytes / Q000201:enzymology*; D008297:Male; D009896:Optic Atrophy / Q000201:enzymology*; D013466:Sulfurtransferases / Q000378:metabolism*; D013884:Thiosulfate Sulfurtransferase / Q000378:metabolism*
D013466:Sulfurtransferases; D013884:Thiosulfate Sulfurtransferase