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Plasma Cholesteryl Sulfate in Friedreich's ataxia

Published online by Cambridge University Press:  18 September 2015

A.C. Nestruck ,
Y.S. Huang ,
K. Eid ,
R. Dufour ,
L. Boulet ,
A. Barbeau  and
J. Davignon
A.C. Nestruck
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
Y.S. Huang
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
K. Eid
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
R. Dufour
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
L. Boulet
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
A. Barbeau*
Affiliation:
Department of Neurobiology, Clinical Research Institute of Montreal
J. Davignon
Affiliation:
Departments of Lipid Metabolism and Atherosclerosis Research, Clinical Research Institute of Montreal
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada, H2W IR7
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Abstract

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Alteration of membrane fluidity and anomalies of membrane structural proteins have been suspected in Friedreich's ataxia. Plasma lecithinxholesterol acyltransferase (LCAT) activity is also lowered in this disease, presumably because of a substrate effect. The membrane-stabilizing effect of cholesteryl sulfate (CS) and its inhibitory effect on LCAT activity prompted us to measure this substance in the plasma of Friedreich's ataxia patients as well as in normal subjects and in patients with Charlevoix-Saguenay disease. Plasma cholesteryl sulfate concentrations were significantly higher in Friedreich's ataxia, with levels above the upper limit of normal in nearly half of the cases. This increase was unrelated to age, sex or plasma cholesterol levels, but closely associated with the severity of the disease and thus considered to be secondary. A similar phenomenon (except the association with severity) was observed in Charlevoix-Saguenay ataxia. Levels also tended to be higher in first-degree relatives of Friedreich cases. The significance of these findings is discussed in the light of recent knowledge and experimental data obtained in this laboratory on rats made deficient in essential fatty acids. The highest concentrations of CS observed in Friedreich's ataxia (1097 µ-g/dL, 6 times the normal mean) was only 25% as high as the concentrations reported to inhibit LCAT activity.

Type
C—Biochemistry
Information
Canadian Journal of Neurological Sciences , Volume 11 , Issue S4 , November 1984 , pp. 631 - 636
Copyright
Copyright © Canadian Neurological Sciences Federation 1984

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