Impairment of P2 Receptor-Mediated Modulation of Skeletal Muscle Contractions in Transgenic Mice with Modeled Amyotrophic Lateral Sclerosis.

We studied the effects of ATP on skeletal muscle contractility in FUS-transgenic mice with amyotrophic lateral sclerosis (ALS) model. Mechanomyography showed that ATP application did not increase the amplitude of electrically induced contractions of the diaphragm muscle, m. extensor digitorum longus, and soleus muscle in FUS-transgenic mice unlike wild-type mice. Application of a non-selective P2 receptor antagonist suramine and application of ATP against the background of suramine did not change the amplitude of contractions of the studied skeletal muscles in FUS-transgenic mice. Thus, FUS model of ALS is based on the impairment of purinergic regulation of skeletal muscle contractile activity, which can play a role in the pathogenesis of ALS.