Behavioral Responses in Animal Model of Congenital Muscular Dystrophy 1D

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• 作者：Clarissa M. Comim ; Aryadnne L. Schactae ; Jaime A. Soares…
• 关键词：Congenital muscular dystrophy 1D ; Large ; Memory and learning ; Behavior ; Mice
• 刊名：Molecular Neurobiology
• 出版年：2016
• 出版时间：January 2016
• 年：2016
• 卷：53
• 期：1
• 页码：402-407
• 全文大小：647 KB
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• 作者单位：Clarissa M. Comim (1)
  Aryadnne L. Schactae (1)
  Jaime A. Soares (1)
  Letícia Ventura (1)
  Viviane Freiberger (1)
  Francielle Mina (2)
  Diogo Dominguini (2)
  Mariz Vainzof (3)
  João Quevedo (2) (4)
  
  1. Laboratory of Experimental Neuropathology, Postgraduate Program in Health Sciences, University of South Santa Catarina, Palhoça, SC, Brazil
  2. Laboratory of Neurosciences, Graduate Program in Health Sciences, Health Sciences Unit, University of Southern Santa Catarina, Criciúma, SC, Brazil
  3. Human Genome Research Center, Biosciences Institute, University of São Paulo, São Paulo, São Paulo, Brazil
  4. Center for Experimental Models in Psychiatry, Department of Psychiatry and Behavioral Sciences, The University of Texas Medical School at Houston, Houston, TX, USA
  
• 刊物主题：Neurosciences; Neurobiology; Cell Biology; Neurology;
• 出版者：Springer US
• ISSN：1559-1182

文摘

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 Largemyd 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 Largemyd 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.