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Decreased plasmalogen ratios in cultured skin fibroblasts from myotonic dystrophy
Author(s) -
Sakai Tetsuo,
Antoku Yasunobu
Publication year - 1987
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.880100707
Subject(s) - myotonic dystrophy , myotonia , plasmalogen , peroxisome , peroxisomal disorder , phytanic acid , intracellular , endocrinology , biochemistry , medicine , lipid metabolism , organelle , chemistry , biology , phospholipid , membrane , gene
It has become known that hypolipidemic agents, which can induce experimental myotonia in humans and animals, may alter the lipid metabolism of intracellular organelles, peroxisomes. However, there has been no reported study on peroxisomal lipid metabolisms in myotonic dystrophy showing myotonia as well as experimental myotonia. We investigated one of the peroxisomal lipid metabolisms, i.e., plasmalogen metabolism in cultured skin fibroblasts from myotonic dystrophy, and found plasmalogen ratios to be significantly reduced.