title: Muscle Expression of SOD1(G93A) Modulates microRNA and mRNA Transcription Pattern Associated with the Myelination Process in the Spinal Cord of Transgenic Mice.
abstract: A crucial system severely affected in several neuromuscular diseases is the loss of effective connection between muscle and nerve, leading to a pathological non-communication between the two tissues. One of the best examples of impaired interplay between muscle and nerve is Amyotrophic Lateral Sclerosis, a neurodegenerative disease characterized by degeneration of motor neurons and muscle atrophy. Increasing evidences suggest that damage to motor neurons is enhanced by alterations in the neighboring non-neuronal cells and indicate that altered skeletal muscle might be the source of signals that impinge motor neuron activity and survival. Here we investigated whether muscle selective expression of SOD1(G93A) mutant gene modulates mRNAs and miRNAs expression at the level of spinal cord of MLC/SOD1(G93A) mice. Using a Taqman array, the Affymetrix Mouse Gene 2.0 ST approach and the MiRwalk 2.0 database, which provides information on miRNA and their predicted target genes, we revealed that muscle specific expression of SOD1(G93A) modulates relevant molecules of the genetic and epigenetic circuitry of myelin homeostasis in spinal cord of transgenic mice. Our study provides insights into the pathophysiological interplay between muscle and nerve and supports the hypothesis that muscle is a source of signals that can either positively or negatively affect the nervous system.
authors.affiliation: DAHFMO-Unit of Histology and Medical Embryology, Institute Pasteur-Cenci Bolognetti, IIM, Sapienza University of Rome Rome, Italy ; Center for Life Nano Science at Sapienza, Istituto Italiano di Tecnologia Rome, Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Dobrowolny
authors.affiliation: Institute of Anatomy and Cell Biology, Università Cattolica del Sacro Cuore Rome, Italy.
authors.forename: Camilla
authors.initials: C
authors.lastname: Bernardini
authors.affiliation: DAHFMO-Unit of Histology and Medical Embryology, Institute Pasteur-Cenci Bolognetti, IIM, Sapienza University of Rome Rome, Italy ; Center for Life Nano Science at Sapienza, Istituto Italiano di Tecnologia Rome, Italy.
authors.forename: Martina
authors.initials: M
authors.lastname: Martini
authors.affiliation: Institute of Anatomy and Cell Biology, Università Cattolica del Sacro Cuore Rome, Italy.
authors.forename: Mirko
authors.initials: M
authors.lastname: Baranzini
authors.affiliation: Institute of Anatomy and Cell Biology, Università Cattolica del Sacro Cuore Rome, Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Barba
authors.affiliation: DAHFMO-Unit of Histology and Medical Embryology, Institute Pasteur-Cenci Bolognetti, IIM, Sapienza University of Rome Rome, Italy ; Center for Life Nano Science at Sapienza, Istituto Italiano di Tecnologia Rome, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Musarò
keywords: ALS; SOD1G93A; miRNA and mRNA signature; muscle-nerve interplay; myelination process