Genetic and acquired defects of lower motor neurons, peripheral nerves, or skeletal muscle are responsible for several neuromuscular disorders. The neuromuscular junction (NMJ) represents the morphofunctional interface between muscle and nerve, and defects in the development and maintenance of the NMJ are responsible for the pathogenesis of several neuromuscular disorders, including congenital myasthenic syndromes, autoimmune myasthenia gravis, spinal muscular atrophy, and amyotrophic lateral sclerosis (ALS); moreover, defects in muscle and nerve communication occur physiologically during aging. The molecular players involved inthe formation and maintenance of theNMJ have been deeply studied; however, the role of skeletal muscle in NMJ dismantlement still needs to be fully elucidated. In this Special Issue, entitled “The Role of Skeletal Muscle in Neuromuscular Diseases: From Cellular and Molecular Players to Therapeutic Interventions”, we collected recent research advances and ongoing studies focused on the muscle denervation associated with aging and neuromuscular diseases. Two of the published papers are focused on the role of skeletal muscle in ALS disease, one on NMJ dismantlement in relation to muscle accumulation of oxidative damage, and the others are focused on the role of skeletal muscle in different diseases, such as muscular dystrophies.
Dobrowolny, G., Scicchitano, B. M., The Role of Skeletal Muscle in Neuromuscular Diseases: From Cellular and Molecular Players to Therapeutic Interventions, <<CELLS>>, 2021; 11 (7): 1207-1211. [doi:10.3390/cells11071207] [http://hdl.handle.net/10807/202642]
The Role of Skeletal Muscle in Neuromuscular Diseases: From Cellular and Molecular Players to Therapeutic Interventions
Scicchitano, Bianca MariaUltimo
2022
Abstract
Genetic and acquired defects of lower motor neurons, peripheral nerves, or skeletal muscle are responsible for several neuromuscular disorders. The neuromuscular junction (NMJ) represents the morphofunctional interface between muscle and nerve, and defects in the development and maintenance of the NMJ are responsible for the pathogenesis of several neuromuscular disorders, including congenital myasthenic syndromes, autoimmune myasthenia gravis, spinal muscular atrophy, and amyotrophic lateral sclerosis (ALS); moreover, defects in muscle and nerve communication occur physiologically during aging. The molecular players involved inthe formation and maintenance of theNMJ have been deeply studied; however, the role of skeletal muscle in NMJ dismantlement still needs to be fully elucidated. In this Special Issue, entitled “The Role of Skeletal Muscle in Neuromuscular Diseases: From Cellular and Molecular Players to Therapeutic Interventions”, we collected recent research advances and ongoing studies focused on the muscle denervation associated with aging and neuromuscular diseases. Two of the published papers are focused on the role of skeletal muscle in ALS disease, one on NMJ dismantlement in relation to muscle accumulation of oxidative damage, and the others are focused on the role of skeletal muscle in different diseases, such as muscular dystrophies.I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.