title: Muscle atrophy is associated with cervical spinal motoneuron loss in BACHD mouse model for Huntington's disease.
abstract: Involuntary choreiform movements are clinical hallmark of Huntington's disease, an autosomal dominant neurodegenerative disorder caused by an increased number of CAG trinucleotide repeats in the huntingtin gene. Involuntary movements start with an impairment of facial muscles and then affect trunk and limbs muscles. Huntington's disease symptoms are caused by changes in cortex and striatum neurons induced by mutated huntingtin protein. However, little is known about the impact of this abnormal protein in spinal cord motoneurons that control movement. Therefore, in this study we evaluated abnormalities in the motor unit (spinal cervical motoneurons, motor axons, neuromuscular junctions and muscle) in a mouse model for Huntington's disease (BACHD). Using light, fluorescence, confocal, and electron microscopy, we showed significant changes such as muscle fibers atrophy, fragmentation of neuromuscular junctions, axonal alterations, and motoneurons death in BACHD mice. Noteworthy, the surviving motoneurons from BACHD spinal cords were smaller than WT. We suggest that this loss of larger putative motoneurons is accompanied by a decrease in the expression of fast glycolytic muscle fibers in this model for Huntington's disease. These observations show spinal cord motoneurons loss in BACHD that might help to understand neuromuscular changes in Huntington's disease.
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Priscila Aparecida Costa
authors.initials: PA
authors.lastname: Valadão
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Bárbara Campos
authors.initials: BC
authors.lastname: de Aragão
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Jéssica Neves
authors.initials: JN
authors.lastname: Andrade
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Matheus Proença S
authors.initials: MP
authors.lastname: Magalhães-Gomes
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Giselle
authors.initials: G
authors.lastname: Foureaux
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Julliane Vasconcelos
authors.initials: JV
authors.lastname: Joviano-Santos
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: José Carlos
authors.initials: JC
authors.lastname: Nogueira
authors.affiliation: Departamento de Bioquímica e Imunologia, ICB, Universidade Federal de Minas Gerais, Belo Horizonte, MG, Brazil.
authors.forename: Fabíola Mara
authors.initials: FM
authors.lastname: Ribeiro
authors.affiliation: Department of Biomedical Sciences, University of Talca, Talca, Chile.
authors.forename: Juan Carlos
authors.initials: JC
authors.lastname: Tapia
authors.affiliation: Departamento de Morfologia, ICB, Universidade Federal de Minas Gerais, Av. Antônio Carlos, 6627, Belo Horizonte, MG, 31270-901, Brazil.
authors.forename: Cristina
authors.identifier: 0000-0001-5392-1266
authors.initials: C
authors.lastname: Guatimosim
mesh_terms: D000818:Animals; D002574:Cervical Vertebrae / Q000473:pathology; D006816:Huntington Disease / Q000473:pathology*; D008297:Male; D051379:Mice; D009046:Motor Neurons / Q000473:pathology*; D018482:Muscle, Skeletal / Q000473:pathology; D009133:Muscular Atrophy / Q000473:pathology*; D009469:Neuromuscular Junction / Q000473:pathology; D013116:Spinal Cord / Q000473:pathology
keywords: BACHD; motoneuron; neuromuscular junctions; spinal cord; sternomastoid muscle