Abstract
Muscle weakness is the main characteristic of amyotrophic lateral sclerosis, which eventually leads to functional impairment and respiratory insufficiency. Progressive weakness derives from a loss of motor units and active muscle fibres. Striated muscle contraction depends on the interaction between intracellular calcium and the protein troponin. Troponin is made up of three subunits—troponin C is especially important for binding calcium and promoting a dynamic interplay between troponin and tropomyosin, with consequent muscle contraction.
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