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  • 1
    ISSN: 1432-1076
    Keywords: Myopathy ; Convulsions ; Mitochondria ; Respiratory chain ; Coenzyme Q
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract A patient is presented who had therapy-resistant epileptic seizures from the 7th day of life. Examination at the age of 17 months revealed a mentally retarded boy with epileptic seizures, generalised myoclonic contractions, and abnormal ocular movements. A cerebral CT scan showed central and cortical atrophy. Lactate levels in serum, cerebrospinal fluid and urine were elevated, the pyruvate level was raised in serum. A quadriceps muscle biopsy revealed aspecific morphologic signs of a myopathy. Biochemical analysis showed decreased substrate oxidation rates in the mitochondria associated with low rates of ATP production. Total and free carnitine levels were decreased. Investigation of the respiratory chain revealed a defect in the proximal part of respiratory chain revealed a defect in the proximal part of respiratory chain involving the region of coenzyme Q. Based on clinical and chemical data it is likely that the patient is suffering from a multi-system disorder.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    New York, NY : Wiley-Blackwell
    International Journal of Chemical Kinetics 20 (1988), S. 775-786 
    ISSN: 0538-8066
    Keywords: Chemistry ; Physical Chemistry
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology
    Notes: In order to bring more information on the thermodynamic and kinetic behavior of nitryl chloride in aprotic media, we have surveyed exhaustively the first NO2Cl-reduction step in sulfolane (at the platinum electrode), taking into account our preliminary results about the electrochemical properties of NO2Cl in aprotic solvents. We have excluded the intervention of the weak ionic dissociation of NO2Cl (NO2Cl ⇆ NO2+ + Cl- [I]) and its slow molecular decomposition as: 2NO2Cl ⇄ Cl2 + 2NO2• (⇄ N2O4) [II] in this process. We have admitted the occurrence of a rapid chemical reaction which controls kinetically the electrochemical system studied: \documentclass{article}\pagestyle{empty}\begin{document}$ {\rm NO} + {\rm NO}_{\rm 2} {\rm Cl}\buildrel {k^*} \over \longrightarrow {\rm NOCl} + {\rm NO}_{\rm 2}^ \cdot {\rm}[{\rm III]} $\end{document}. By analyzing the kinetic currents resulting from the 1 st cathodic wave of NO2Cl at the temperature range 303-323 K, the rate constant, k*, and the activation energy, E*, of reaction [III] have been determined. These results and those previously found in the gas phase are discussed.
    Additional Material: 4 Ill.
    Type of Medium: Electronic Resource
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