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Alterations in fast‐twitch muscle membrane conductance regulation do not explain decreased muscle function of SOD1G93A rats

肌萎缩侧索硬化 SOD1 电生理学 神经肌肉传递 神经肌肉接头 复合肌肉动作电位 运动神经元 骨骼肌 电机单元 神经科学 医学 内科学 解剖 化学 内分泌学 生物 疾病
作者
Pieter A. Leermakers,Martin Skov,Anders Riisager,Ole Bækgaard Nielsen,Thomas Holm Pedersen
出处
期刊:Muscle & Nerve [Wiley]
卷期号:64 (6): 755-764 被引量:1
标识
DOI:10.1002/mus.27413
摘要

Abstract Introduction/Aims Both neuromuscular junction (NMJ) dysfunction and altered electrophysiological properties of muscle fibers have been reported in amyotrophic lateral sclerosis (ALS) patients. ALS‐related preclinical studies typically use rodent SOD1 G93A overexpression models, but translation to the human disease has been challenged. The present work explored NMJ function and cellular electrophysiological properties of muscles fibers in SOD1 G93A overexpression rats. Methods Longitudinal studies of compound muscle action potentials (CMAPs) were performed in SOD1 G93A rats. Cellular studies were performed to evaluate electrophysiological properties of muscle fibers, including the resting membrane conductance ( G m ) and its regulation during prolonged action potential (AP) firing. Results SOD1 G93A rats showed a substantial loss of gastrocnemius CMAP amplitude (35.8 mV, P < .001) and a minor increase in CMAP decrement (8.5%, P = .002) at 25 weeks. In addition, SOD1 G93A EDL muscle fibers showed a lower baseline G m (wild‐type, 1325 μS/cm 2 ; SOD1 G93A , 1137 μS/cm 2 ; P < .001) and minor alterations in G m regulation during repeated firing of APs as compared with wild‐type rats. Discussion The current data suggest that loss of CMAP amplitude is largely explained by defects in either lower motor neuron or skeletal muscle with only minor indications of a role for neuromuscular transmission defects in SOD1 G93A rats. Electrophysiological properties of muscle fibers were not markedly affected, and an elevated G m , as has been reported in motor neuron disease (MND) patients, was not replicated in SOD1 G93A muscles. Collectively, the neuromuscular pathology of SOD1 G93A rats appears to differ from that of ALS/MND patients with respect to neuromuscular transmission defects and electrophysiological properties of muscle fibers.
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