Diazacholesterol Myotonia: Accumulation of Desmosterol and Increased Adenosine Triphosphatase Activity of Sarcolemma
James B. Peter 1 and
Walter Fiehn 1
1 Neuromuscular Disease Research Laboratories, Department of Medicine, University of California School of Medicine, Los Angeles 90024
Myotonia induced in rats by 20,25-diazacholesterol is accompanied by accumulation of desmosterol in serum, fragmented sarcoplasmic reticulum, and sarcolemma. Activities of (Na+,K+)- and Ca2+-stimulated adenosine triphosphatases of the sarcolemma are increased, but not the Mg2+-stimulated adenosine triphosphatase. The altered sterol composition of the sarcolemma may cause this type of myotonia by decreasing the chloride conductance of the membrane.