Takagi A, Kojima S, Ida M, Araki M
Department of Neurology, Toranomon Hospital, Tokyo, Japan.
J Neurol Sci. 1992 Jul;110(1-2):160-4. doi: 10.1016/0022-510x(92)90023-e.
Using a single skinned muscle fiber, the function of the contractile system and the sarcoplasmic reticulum (SR) were analyzed in the skeletal muscle of the mdx mouse. Activation of the contractile system by calcium ion and the maximum force generation was normal. Ca2+ uptake of the SR was normal as well as regulation of the Ca-induced Ca release (CICR) by Ca2+. However, contracture by caffeine was more prominent in mdx than in control mice. Ca2+ leaked more from the SR of mdx in the presence of EGTA and ATP or its analogue. These abnormalities are probably interrelated; increased leakage of Ca2+ might cause the enhanced response to caffeine, since Ca2+ itself facilitates Ca release by caffeine. The abnormal leakage of Ca2+ might also activate the Ca pump of SR in the resting state, which consume extra ATP and disturb energy metabolism.
利用单根去表皮肌肌肌肌纤维,对mdx小鼠骨骼肌中收缩系统和肌浆网(SR)的功能进行了分析。钙离子对收缩系统的激活以及最大力量产生均正常。SR的Ca2+摄取以及Ca2+对钙诱导钙释放(CICR)的调节也正常。然而,mdx小鼠中咖啡因引起的挛缩比对照小鼠更明显。在存在乙二醇双四乙酸(EGTA)和三磷酸腺苷(ATP)或其类似物的情况下,mdx小鼠的SR中Ca2+泄漏更多。这些异常情况可能相互关联;Ca2+泄漏增加可能导致对咖啡因的反应增强,因为Ca2+本身可促进咖啡因诱导的钙释放。Ca2+的异常泄漏还可能在静息状态下激活SR的钙泵,这会消耗额外的ATP并干扰能量代谢。