Minou Verhaeg, Kevin Adamzek, Davy van de Vijver, Kayleigh Putker, Sarah Engelbeen, Daphne Wijnbergen, Maurice Overzier, Ernst Suidgeest, Louise van der Weerd, Annemieke Aartsma-Rus, Maaike van Putten
{"title":"Learning, memory and blood–brain barrier pathology in Duchenne muscular dystrophy mice lacking Dp427, or Dp427 and Dp140","authors":"Minou Verhaeg, Kevin Adamzek, Davy van de Vijver, Kayleigh Putker, Sarah Engelbeen, Daphne Wijnbergen, Maurice Overzier, Ernst Suidgeest, Louise van der Weerd, Annemieke Aartsma-Rus, Maaike van Putten","doi":"10.1111/gbb.12895","DOIUrl":null,"url":null,"abstract":"<p>Duchenne muscular dystrophy is a severe neuromuscular disorder that is caused by mutations in the <i>DMD</i> gene, resulting in a disruption of dystrophin production. Next to dystrophin expression in the muscle, different isoforms of the protein are also expressed in the brain and lack of these isoforms leads to cognitive and behavioral deficits in patients. It remains unclear how the loss of the shorter dystrophin isoform Dp140 affects these processes. Using a variety of behavioral tests, we found that <i>mdx</i> and <i>mdx</i><sup><i>4cv</i></sup> mice (which lack Dp427 or Dp427 + Dp140, respectively) exhibit similar deficits in working memory, movement patterns and blood–brain barrier integrity. Neither model showed deficits in spatial learning and memory, learning flexibility, anxiety or spontaneous behavior, nor did we observe differences in aquaporin 4 and glial fibrillary acidic protein. These results indicate that in contrast to Dp427, Dp140 does not play a crucial role in processes of learning, memory and spontaneous behavior.</p>","PeriodicalId":50426,"journal":{"name":"Genes Brain and Behavior","volume":"23 3","pages":""},"PeriodicalIF":2.4000,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/gbb.12895","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Genes Brain and Behavior","FirstCategoryId":"102","ListUrlMain":"https://onlinelibrary.wiley.com/doi/10.1111/gbb.12895","RegionNum":4,"RegionCategory":"心理学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q2","JCRName":"BEHAVIORAL SCIENCES","Score":null,"Total":0}
引用次数: 0
Abstract
Duchenne muscular dystrophy is a severe neuromuscular disorder that is caused by mutations in the DMD gene, resulting in a disruption of dystrophin production. Next to dystrophin expression in the muscle, different isoforms of the protein are also expressed in the brain and lack of these isoforms leads to cognitive and behavioral deficits in patients. It remains unclear how the loss of the shorter dystrophin isoform Dp140 affects these processes. Using a variety of behavioral tests, we found that mdx and mdx4cv mice (which lack Dp427 or Dp427 + Dp140, respectively) exhibit similar deficits in working memory, movement patterns and blood–brain barrier integrity. Neither model showed deficits in spatial learning and memory, learning flexibility, anxiety or spontaneous behavior, nor did we observe differences in aquaporin 4 and glial fibrillary acidic protein. These results indicate that in contrast to Dp427, Dp140 does not play a crucial role in processes of learning, memory and spontaneous behavior.
期刊介绍:
Genes, Brain and Behavior was launched in 2002 with the aim of publishing top quality research in behavioral and neural genetics in their broadest sense. The emphasis is on the analysis of the behavioral and neural phenotypes under consideration, the unifying theme being the genetic approach as a tool to increase our understanding of these phenotypes.
Genes Brain and Behavior is pleased to offer the following features:
8 issues per year
online submissions with first editorial decisions within 3-4 weeks and fast publication at Wiley-Blackwells
High visibility through its coverage by PubMed/Medline, Current Contents and other major abstracting and indexing services
Inclusion in the Wiley-Blackwell consortial license, extending readership to thousands of international libraries and institutions
A large and varied editorial board comprising of international specialists.