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NEUROLOGY 1987;37:1878
© 1987 American Academy of Neurology

Normal rhodanese activity in leukocytes from Leber patients

Enzyme characterization and activity levels

R. Pallini, MD, P. Martelli, DrPh, A. M. Bardelli, MD, G. C. Guazzi, MD and A. Federico, MD

Istituto di Scienze Neurologiche e Centro per lo Studio delle Encefalo-Neuro-Miopatie Genetiche (Drs. Pallini, Guazzi, and Federico), Istituto di Chimica Biologica (Dr. Martelli), and the Istituto di Scienze Oftalmologiche (Dr, Bardelli), Università di Siena, Siena, Italy.

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 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 x 10–3 M. No significant difference of enzyme activity was present in Leber's disease.

Address correspondence and reprint requests to Dr. Federico, Istituto di Scienze Neurologiche, Policlinico Universitario, Viale Bracci, 53100 Siena, Italy.

Supported by a grant from CNR (Roma), Progetto Finalizzato Ingegneria Genetica e Basi Molecolari delle Malattie Ereditarie e Malattie del Sistema Nervoso.

Presented in part at the thirty-eighth annual meeting of the American Academy of Neurology, New Orleans, LA, April 1986.

Received October 8, 1986. Accepted for publication in final form March 2, 1987.






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