title: Behavioral Responses in Animal Model of Congenital Muscular Dystrophy 1D.
abstract: Congenital muscular dystrophies 1D (CMD1D) present a mutation on the LARGE gene and are characterized by an abnormal glycosylation of α-dystroglycan (α-DG), strongly implicated as having a causative role in the development of central nervous system abnormalities such as cognitive impairment seen in patients. However, in the animal model of CMD1D, the brain involvement remains unclear. Therefore, the objective of this study is to evaluate the cognitive involvement in the Large(myd) mice. To this aim, we used adult homozygous, heterozygous, and wild-type mice. The mice underwent six behavioral tasks: habituation to an open field, step-down inhibitory avoidance, continuous multiple trials step-down inhibitory avoidance task, object recognition, elevated plus-maze, and forced swimming test. It was observed that Large(myd) individuals presented deficits on the habituation to the open field, step down inhibitory avoidance, continuous multiple-trials step-down inhibitory avoidance, object recognition, and forced swimming. This study shows the first evidence that abnormal glycosylation of α-DG may be affecting memory storage and restoring process in an animal model of CMD1D.
authors.affiliation: Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil. clarissamc@gmail.com.
authors.forename: Clarissa M
authors.initials: CM
authors.lastname: Comim
authors.affiliation: Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil.
authors.forename: Aryadnne L
authors.initials: AL
authors.lastname: Schactae
authors.affiliation: Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil.
authors.forename: Jaime A
authors.initials: JA
authors.lastname: Soares
authors.affiliation: Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil.
authors.forename: Letícia
authors.initials: L
authors.lastname: Ventura
authors.affiliation: Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil.
authors.forename: Viviane
authors.initials: V
authors.lastname: Freiberger
authors.affiliation: Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit, University of Southern Santa Catarina, Criciúma, SC, Brazil.
authors.forename: Francielle
authors.initials: F
authors.lastname: Mina
authors.affiliation: Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit, University of Southern Santa Catarina, Criciúma, SC, Brazil.
authors.forename: Diogo
authors.initials: D
authors.lastname: Dominguini
authors.affiliation: Human Genome Research Center, Biosciences Institute, University of São Paulo, São Paulo, São Paulo, Brazil.
authors.forename: Mariz
authors.initials: M
authors.lastname: Vainzof
authors.affiliation: Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit, University of Southern Santa Catarina, Criciúma, SC, Brazil.|Center for Experimental Models in Psychiatry, Department of Psychiatry and Behavioral Sciences, The University of Texas Medical School at Houston, Houston, TX, USA.
authors.forename: João
authors.initials: J
authors.lastname: Quevedo
mesh_terms: D000818:Animals; D001362:Avoidance Learning; D001522:Behavior, Animal; D004195:Disease Models, Animal; D006185:Habituation, Psychophysiologic; D018782:Maze Learning; D018345:Mice, Knockout; D009137:Muscular Dystrophy, Animal / Q000151:congenital* / Q000473:pathology* / Q000503:physiopathology; D013550:Swimming
keywords: Behavior; Congenital muscular dystrophy 1D; Large; Memory and learning; Mice