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Biochemical and ultrastructural features of human fibroblasts cultured from a new variant of type 3 lipid storage myopathy
Author(s) -
Radom Jean,
Salvayre Robert,
Mussini JeanMarie,
Lisle Bernadette,
Negre Anne,
Maret Arlette,
Billaudel Sylviane,
DousteBlazy Louis
Publication year - 1988
Publication title -
biology of the cell
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 85
eISSN - 1768-322X
pISSN - 0248-4900
DOI - 10.1111/j.1768-322x.1988.tb00704.x
Subject(s) - biology , lipid droplet , vacuole , nile red , biochemistry , cytoplasm , myopathy , nile blue , ultrastructure , oleic acid , endogeny , lipid metabolism , carnitine , microbiology and biotechnology , anatomy , fluorescence , physics , genetics , quantum mechanics
A new variant of multisystemic lipid storage myopathy (type 3) has been identified. Human cultured fibroblasts present a major triacylglycerol storage whereas other neutral lipids and phospholipids are in the normal range. When feeding the cells in the presence of radiolabelled oleic acid we observed an accumulation of radiolabelled triacylglycerols demonstrating the endogenous biosynthesis of the stored triacylglycerols. After a 72‐hr chase period, no degradation of radiolabelled triacylglycerols was observed. Histochemical examination of multisystemic lipid storage myopathy skin fibroblasts showed a massive accumulation of neutral lipids (stained by the fluorescent probe Nile Red) in cells grown in medium supplemented with 10% fetal calf serum. These cytoplasmic vacuoles were not obviously membrane‐surrounded as shown by electron microscopy.