Normal rhodanese activity in leukocytes from Leber patients
- 1 December 1987
- journal article
- research article
- Published by Wolters Kluwer Health in Neurology
- Vol. 37 (12) , 1878
- https://doi.org/10.1212/wnl.37.12.1878
Abstract
The thiosulfate: cyanide sulfurtransferase (rhodanese) enzyme (EC 2.8.1.1.) was studied in human leukocytes from control subjects and from nine patients with Leber9s hereditan 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 × 10−3 M. No significant difference of enzyme activity was present in Leber9s disease.This publication has 3 references indexed in Scilit:
- Deficiency of thiosulphate sulphurtransferase (rhodanese) in Leber's hereditary optic neuropathy.BMJ, 1986
- Mitochondrial Inheritance in a Mitochondrially Mediated DiseaseNew England Journal of Medicine, 1983
- Rhodanese‐Mediated Sulfur Transfer to Succinate DehydrogenaseEuropean Journal of Biochemistry, 1977