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Title: Normal composition of the major lipids and the distribution of their fatty acids in central nervous system myelin further discriminate mld from shiverer mice. Author: Fressinaud C, Sarliève LL, Cassagne C, Vincendon G, Matthieu JM. Journal: Dev Neurosci; 1990; 12(1):61-72. PubMed ID: 1688761. Abstract: The primary defect in myelin deficiency (mld), an autosomal recessive mutation in mice with severe hypomyelination of the central nervous system (CNS), is a reduction in the synthesis of myelin basic protein (MBP) due to reduced amounts of MBP-specific mRNA. The present study was performed to determine whether alterations of myelin lipid composition might be associated with this defect. Although a 20-fold reduction of myelin was found in mld brain, the lipid/protein and the phospholipid/sulfatide ratios of purified myelin were unchanged. The analysis of polar lipids demonstrated no striking difference. However, phosphatidylethanolamine (including plasmalogens of the ethanolamine type) was decreased by 7.7% in mld. Fatty acids of total and polar lipids (phospholipids + glycolipids) exhibited a slight decrease in 20:1(n-11) and 20:1(n-9). In conclusion, these results further differentiate mld from its allele shiverer, which shares with mld a dramatic reduction of MBP and absence of major dense line but, in contrast, presents other important biochemical differences in CNS myelin.[Abstract] [Full Text] [Related] [New Search]