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Absence Of Physiological Ca2+ Transients Is An Initial Trigger For Mitochondrial Dysfunction In Skeletal Muscle Following Denervation, Chehade Karam, Jianxun Yi, Yajuan Xiao, Kamal Dhakal, Lin Zhang, Xuejun Li, Carlo Manno, Jiejia Xu, Kaitao Li, Heping Cheng, Jianjie Ma, Jingsong Zhou

Faculty Publications

Background: Motor neurons control muscle contraction by initiating action potentials in muscle. Denervation of muscle from motor neurons leads to muscle atrophy, which is linked to mitochondrial dysfunction. It is known that denervation promotes mitochondrial reactive oxygen species (ROS) production in muscle, whereas the initial cause of mitochondrial ROS production in denervated muscle remains elusive. Since denervation isolates muscle from motor neurons and deprives it from any electric stimulation, no action potentials are initiated, and therefore, no physiological Ca2+ transients are generated inside denervated muscle fibers. We tested whether loss of physiological Ca2+ transients is an initial cause leading to …

Ivv-13 Beneficial Effect Of Oxaloacetate For The Neuromuscular Function Of Sod1g93a Mice, Hiroshi Nishimune, Sudheer K. Tungtur, Heather M. Wilkins, Russell H. Swerdlow, Jessica Sage, Abdulbaki Agbas, Richard J. Barohn

Faculty Publications

Introduction and aims: The etiology of ALS remains unknown despite the identification of mutated genes in familial and/or sporadic ALS. It is thought that aggregates of disease-causing mutant proteins and RNAs disrupt the normal cellular functions that leads to dying back neuropathy and motor neuron degeneration. Mitochondrial dysfunction is thought to play a major role in the pathology of ALS (1–3). Therefore, restoration of mitochondrial function is likely to cause beneficial effects for the survival of ALS model mice (4).

The objective of this project is to evaluate the effects of oxaloacetate for the neuromuscular function and lifespan of the …