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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 <jats:styled-content style=\"fixed-case\">BACHD<\/jats:styled-content> mice. Noteworthy, the surviving motoneurons from <jats:styled-content style=\"fixed-case\">BACHD<\/jats:styled-content> spinal cords were smaller than <jats:styled-content style=\"fixed-case\">WT<\/jats:styled-content>. 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 <jats:styled-content style=\"fixed-case\">BACHD<\/jats:styled-content> that might help to understand neuromuscular changes in Huntington's disease.<\/jats:p>","DOI":"10.1111\/ejn.13510","type":"journal-article","created":{"date-parts":[[2016,12,19]],"date-time":"2016-12-19T14:46:58Z","timestamp":1482158818000},"page":"785-796","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Muscle atrophy is associated with cervical spinal motoneuron loss in BACHD mouse model for Huntington's disease"],"prefix":"10.1111","volume":"45","author":[{"given":"Priscila Aparecida Costa","family":"Valad\u00e3o","sequence":"first","affiliation":[{"name":"Departamento de Morfologia, ICB Universidade Federal de Minas Gerais Av. 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