Please use this identifier to cite or link to this item: http://hdl.handle.net/10397/61901
Title: Hippocampal dysfunction and cognitive impairment in fragile-x syndrome
Authors: Bostrom, C
Yau, SY 
Majaess, N
Vetrici, M
Gil-Mohapel, J
Christie, BR
Keywords: Fmr1 knock-out mice
Fragile-X Syndrome
Hippocampus
MGluR theory
NMDA receptor
Synaptic plasticity
Issue Date: 2016
Publisher: Pergamon
Source: Neuroscience & biobehavioral reviews, 2016, v. 68, p. 563-574 How to cite?
Journal: Neuroscience & biobehavioral reviews 
Abstract: Fragile-X Syndrome (FXS) is the most common form of inherited intellectual disability and the leading genetic cause of autism spectrum disorder. FXS is caused by transcriptional silencing of the Fragile X Mental Retardation 1 (Fmr1) gene due to a CGG repeat expansion, resulting in the loss of Fragile X Mental Retardation Protein (FMRP). FMRP is involved in transcriptional regulation and trafficking of mRNA from the nucleus to the cytoplasm and distal sites both in pre- and post-synaptic terminals. Consequently, FXS is a multifaceted disorder associated with impaired synaptic plasticity. One region of the brain that is significantly impacted by the loss of FMRP is the hippocampus, a structure that plays a critical role in the regulation of mood and cognition. This review provides an overview of the neuropathology of Fragile-X Syndrome, highlighting how structural and synaptic deficits in hippocampal subregions, including the CA1 exhibiting exaggerated metabotropic glutamate receptor dependent long-term depression and the dentate gyrus displaying hypofunction of N-methyl-D-aspartate receptors, contribute to cognitive impairments associated with this neurodevelopmental disorder.
URI: http://hdl.handle.net/10397/61901
ISSN: 0149-7634
DOI: 10.1016/j.neubiorev.2016.06.033
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