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Keywords:

  • ALS;
  • motor unit;
  • contractile properties;
  • EMG;
  • transgenic model

ABSTRACT

Introduction: In amyotrophic lateral sclerosis (ALS), progressive death of motor neurons results in denervation and reinnervation of muscles. It is not clear how ALS affects the properties of motor units (MUs). Methods: Properties of single MUs in the medial gastrocnemius (MG) muscle of rats bearing the human mutated superoxide dismutase gene type 1 (SOD1) were determined at 3 stages: asymptomatic (ALS I); early symptomatic (ALS II); and terminal (ALS III). Results: In ALS II, higher proportions of FF (fast fatigable) and S (slow) MUs were observed, whereas in ALS III higher percentages of S and lower percentages of FF MUs were noted compared with controls. S motor neurons reinnervated fast muscle fibers, and those MUs gained some properties of fast MUs, including lower fatigue resistance, greater force generation, and higher action potential amplitudes. Conclusion: Changes in MU properties of SOD1 rats have progressive and multidirectional character and speed depending on the MU type. Muscle Nerve 50: 577–586, 2014