Abnormal fatty acid composition in sarcolemma and sarcoplasmic reticulum from myotonic ADR mouse muscle

Abstract

The fatty acid composition of membrane lipids from sarcolemma and sarcoplasmic reticulum isolated from biceps and gastrocnemius muscle has been compared in normal (wildtype, +/adr mto or +/+) and affected (adr mto/adr mto) myotonic mice. The adr mto mouse exhibits an arrested development of the righting response, and arose spontaneously from the SWR/J strain. These mice exhibit classical myotonia similar to the human disease, Becker's myotonia [1]. Significant alterations, characterized by a decrease in the saturated fatty acid, palmitic acid (16:0), and the polyunsaturated fatty acid, arachidonic acid (20:4), and an increase in stearic (18:0) and linoleic (18:2) acids, were observed between sarcolemma and sarcoplasmic reticulum from normal and affected mice. These changes in fatty acid composition of muscle membrane from ADR mice may be adequate to cause an alteration in membrane fluidity and affect the function of ion channels. The fatty acid composition of erythrocytes ghosts was also examined, as a potential marker for alterations in muscle membranes. In erythrocyte ghosts isolated from affected mice, the only alteration observed was a decrease in the proportion of oleic acid (18:1), an effect completely different from those observed in muscle membranes. Therefore, erythrocyte ghosts do not serve as an adequate indicator of changes in fatty acid composition of muscle membranes in this model of myotonia.