缺乏Dp427或Dp427和Dp140的杜氏肌营养不良症小鼠的学习、记忆和血脑屏障病理变化

IF 2.4 4区 心理学 Q2 BEHAVIORAL SCIENCES
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
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引用次数: 0

摘要

杜兴氏肌肉萎缩症是一种严重的神经肌肉疾病,由 DMD 基因突变引起,导致肌营养不良。除了在肌肉中表达肌营养不良蛋白外,该蛋白的不同异构体也在大脑中表达,缺乏这些异构体会导致患者出现认知和行为障碍。目前仍不清楚较短的肌营养不良蛋白异构体 Dp140 的缺失会如何影响这些过程。通过各种行为测试,我们发现 mdx 和 mdx4cv 小鼠(分别缺乏 Dp427 或 Dp427 + Dp140)在工作记忆、运动模式和血脑屏障完整性方面表现出相似的缺陷。这两种模型在空间学习和记忆、学习灵活性、焦虑或自发行为方面都没有表现出缺陷,我们也没有观察到水通道蛋白 4 和神经胶质纤维酸性蛋白的差异。这些结果表明,与 Dp427 不同,Dp140 在学习、记忆和自发行为过程中并不发挥关键作用。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Learning, memory and blood–brain barrier pathology in Duchenne muscular dystrophy mice lacking Dp427, or Dp427 and Dp140

Learning, memory and blood–brain barrier pathology in Duchenne muscular dystrophy mice lacking Dp427, or Dp427 and Dp140

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.

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来源期刊
Genes Brain and Behavior
Genes Brain and Behavior 医学-行为科学
CiteScore
6.80
自引率
4.00%
发文量
62
审稿时长
4-8 weeks
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.
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