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Hypertriglyceridemia and Lower LDL Cholesterol Concentration in Relation to Apolipoprotein E Phenotypes in Myotonic Dystrophy

Published online by Cambridge University Press:  18 September 2015

Sital Moorjani ,
Daniel Gaudet ,
Claude Laberge ,
Marie Christine Thibault ,
Jean Mathieu ,
Jean Morissette ,
Paul J. Lupien ,
Daniel Brun  and
Claude Gagné
Sital Moorjani*
Affiliation:
Lipid Research Unit, Le Centre Hospitalier de l'Université Laval
Daniel Gaudet
Affiliation:
Human Genetics, Le Centre Hospitalier de l'Université Laval
Claude Laberge
Affiliation:
Human Genetics, Le Centre Hospitalier de l'Université Laval
Marie Christine Thibault
Affiliation:
Human Genetics, Le Centre Hospitalier de l'Université Laval
Jean Mathieu
Affiliation:
Human Genetics, Le Centre Hospitalier de l'Université Laval
Jean Morissette
Affiliation:
Muscular Dystrophy Clinic, Hôpital de Chicoutimi
Paul J. Lupien
Affiliation:
Lipid Research Unit, Le Centre Hospitalier de l'Université Laval
Daniel Brun
Affiliation:
Lipid Research Unit, Le Centre Hospitalier de l'Université Laval
Claude Gagné
Affiliation:
Lipid Research Unit, Le Centre Hospitalier de l'Université Laval
*
Lipid Research Unit, Le Centre Hospitalier de l'Université Laval, 2705 Boulevard Laurier, Sainte-Foy, Québec, Canada G1V 4G2
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Abstract:

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Plasma lipid, lipoprotein levels and apolipoprotein apo E phenotypes were determined in 70 patients with myotonic dystrophy (MyD) and 81 controls. Marked differences were noticed in the apo E phenotype frequencies between the two groups. Plasma triglycerides and VLDL cholesterol were higher in MyD than controls, but only the latter was related to differences in the apo E phenotypes between two groups. Accordingly, the ratio of VLDL cholesterol/plasma triglycerides was increased significantly in MyD, suggesting accumulation of intermediary density particles due to lower affinity of E2 containing lipoproteins for lipoprotein cell receptors. The LDL cholesterol concentration was lower in MyD than controls and was related to differences in the apo E phenotype frequencies between the two groups. These results indicate increased removal of LDL particles in the apo E2 phenotypes, perhaps due to upregulation of LDL (B, E) receptor activity. Plasma cholesterol and HDL cholesterol concentrations were similar in both groups. Another feature of the study was lower levels of plasma cholesterol, triglycerides, VLDL and LDL cholesterol in the homozygous E4:E4 phenotype. These results suggest increased clearance rate of both VLDL and LDL particles and support the concept that apo E4-containing lipoproteins have higher in vivo affinity for ape E and/or B, E receptors.

Type
SPECIAL SUPPLEMENT Dystrophie myotonique au Québec
Information
Canadian Journal of Neurological Sciences , Volume 16 , Issue 1 , February 1989 , pp. 129 - 133
Copyright
Copyright © Canadian Neurological Sciences Federation 1989

References

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