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Plasma Lipids and Lipoproteins in Friedreich's Ataxia and Familial Spastic Ataxia — Evidence for an Abnormal Composition of High Density Lipoproteins

Published online by Cambridge University Press:  18 September 2015

Y. S. Huang ,
A. C. Nestruck ,
A. Barbeau ,
J. P. Bouchard  and
J. Davignon
Y. S. Huang
Affiliation:
Clinical Research Institute of Montreal and l'Hôpital de l'Enfant Jésus, Quebec
A. C. Nestruck
Affiliation:
Clinical Research Institute of Montreal and l'Hôpital de l'Enfant Jésus, Quebec
A. Barbeau*
Affiliation:
Clinical Research Institute of Montreal and l'Hôpital de l'Enfant Jésus, Quebec
J. P. Bouchard
Affiliation:
Clinical Research Institute of Montreal and l'Hôpital de l'Enfant Jésus, Quebec
J. Davignon
Affiliation:
Clinical Research Institute of Montreal and l'Hôpital de l'Enfant Jésus, Quebec
*
Clinical Research Institute of Montreal, 110 Pine Avenue West, Montreal, Quebec, Canada, H2W 1R7
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A systematic study of plasma lipids and lipoproteins was carried out in II cases of Friedreich's ataxia and 6 cases of familial spastic ataxia (Charlevoix-Saguenay disease) using II healthy normolipidemic volunteers of comparable age and sex as controls. No differences were noted in the fatty acid profile of the total lipid fraction, in the total cholesterol and phospholipids or in the percentage distribution of the individual phospholipid classes. The triglycerides were significantly higher in Friedreich's ataxia, but remained within the normal range. Although no systematic abnormalities could be detected in the electrophoretic pattern of plasma lipoproteins or in the apolipoprotein profile on polyacrylamide gel electrophoresis, major differences were found in the high density lipoprotein (HDL) fraction. Their total amount was reduced and their composition was abnormal in both neurological diseases. In Friedreich patients, the relative proportion of cholesterol and triglycerides was increased while the relative protein content was greatly reduced. In Charlevoix disease, a similar abnormality was seen except for the excess of triglycerides. The proportion of phospholipids in HDL was the same in the three groups of patients. In addition, the low density lipoprotein (LDL) fraction was slightly reduced in both diseases. This anomaly of the HDL fraction could indicate that the HDL apolipoprotein moiety has a greater affinity for cholesterol and triglycerides in Friedreich's ataxia than its normal counterpart.

Type
Quebec Cooperative Study of Friedreich's Ataxia
Information
Canadian Journal of Neurological Sciences , Volume 5 , Issue 1 , February 1978 , pp. 149 - 156
Copyright
Copyright © Canadian Neurological Sciences Federation 1978

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