The Keio Journal of Medicine
Online ISSN : 1880-1293
Print ISSN : 0022-9717
ISSN-L : 0022-9717
SERUM DESMOSTEROL AND OTHER LIPIDS IN MYOTONIC DYSTROPHY
A POSSIBLE PATHOGENESIS OF MYOTONIC DYSTROPHY
HIDEO WAKAMATSUHARUO NAKAMURAKIYOAKI ITOWAKO ANAZAWASHIGETAKA OKAJIMASHIGEMICHI OKAMOTOKOJI SHIGENOYUICHIRO GOTO
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1970 Volume 19 Issue 3 Pages 145-149

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Abstract

It has been reported that several inhibitors of cholesterol biosynthesis can induce myotonia, cataract and baldness, which are frequently observed in myotonic dystrophy, in man and experimental animals with concomitant accu-mulation of desmosterol.
These observations suggest that the abnormal lipid metabolism play a pathogenetic role in myotonic dystrophy.
The results of this study showed significantly elevated serum desmosterol, total cholesterol and triglyceride in myotonic dystrophy, indicating a possibility of pathogenetic role of abnormal lipid metabolism in this disease.

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