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Sci Rep. 2018 Jun 11;8(1):8889. doi: 10.1038/s41598-018-26853-z.

Impaired hippocampal representation of place in the Fmr1-knockout mouse model of fragile X syndrome.

Author information

1
Cognitive and Systems Neuroscience, Swammerdam Institute, Center for Neuroscience, Faculty of Science, University of Amsterdam, Science Park 904, 1098 XH, Amsterdam, The Netherlands. tara.arbab@gmail.com.
2
Netherlands Institute for Neuroscience, Institute of the Royal Netherlands Academy of Arts and Sciences, Meibergdreef 47, 1105 BA, Amsterdam, The Netherlands. tara.arbab@gmail.com.
3
Department of Psychiatry, Academic Medical Center, University of Amsterdam, Postal Box 22660, 1100 DD, Amsterdam, The Netherlands. tara.arbab@gmail.com.
4
Cognitive and Systems Neuroscience, Swammerdam Institute, Center for Neuroscience, Faculty of Science, University of Amsterdam, Science Park 904, 1098 XH, Amsterdam, The Netherlands.
5
Research Priority Program Brain and Cognition, University of Amsterdam, Postal Box 94216, 1090 GE, Amsterdam, The Netherlands.
6
Donders Institute for Brain, Cognition, and Behaviour, Radboud Universiteit Nijmegen, Heyendaalseweg 135, 6525 AJ, Nijmegen, The Netherlands.

Abstract

Fragile X syndrome (FXS) is an X-chromosome linked intellectual disability and the most common known inherited single gene cause of autism spectrum disorder (ASD). Building upon demonstrated deficits in neuronal plasticity and spatial memory in FXS, we investigated how spatial information processing is affected in vivo in an FXS mouse model (Fmr1-KO). Healthy hippocampal neurons (so-called place cells) exhibit place-related activity during spatial exploration, and their firing fields tend to remain stable over time. In contrast, we find impaired stability and reduced specificity of Fmr1-KO spatial representations. This is a potential biomarker for the cognitive dysfunction observed in FXS, informative on the ability to integrate sensory information into an abstract representation and successfully retain this conceptual memory. Our results provide key insight into the biological mechanisms underlying cognitive disabilities in FXS and ASD, paving the way for a targeted approach to remedy these.

PMID:
29892074
PMCID:
PMC5995880
DOI:
10.1038/s41598-018-26853-z
[Indexed for MEDLINE]
Free PMC Article

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