scholarly journals Abnormal intrinsic dynamics of dendritic spines in a fragile X syndrome mouse model in vivo

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Akira Nagaoka ◽  
Hiroaki Takehara ◽  
Akiko Hayashi-Takagi ◽  
Jun Noguchi ◽  
Kazuhiko Ishii ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Dendritic Spines ◽  
Fragile X

scholarly journals Restoration of FMRP expression in adult V1 neurons rescues visual deficits in a mouse model of fragile X syndrome

2021 ◽  
Author(s):  
Chaojuan Yang ◽  
Yonglu Tian ◽  
Feng Su ◽  
Yangzhen Wang ◽  
Mengna Liu ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Visual Processing ◽  
Fragile X ◽  
Autism Spectrum ◽  
Inhibitory Neurons ◽  
Local Network ◽  
V1 Neurons ◽  
Processing Deficits

AbstractMany people affected by fragile X syndrome (FXS) and autism spectrum disorders have sensory processing deficits, such as hypersensitivity to auditory, tactile, and visual stimuli. Like FXS in humans, loss of Fmr1 in rodents also cause sensory, behavioral, and cognitive deficits. However, the neural mechanisms underlying sensory impairment, especially vision impairment, remain unclear. It remains elusive whether the visual processing deficits originate from corrupted inputs, impaired perception in the primary sensory cortex, or altered integration in the higher cortex, and there is no effective treatment. In this study, we used a genetic knockout mouse model (Fmr1KO), in vivo imaging, and behavioral measurements to show that the loss of Fmr1 impaired signal processing in the primary visual cortex (V1). Specifically, Fmr1KO mice showed enhanced responses to low-intensity stimuli but normal responses to high-intensity stimuli. This abnormality was accompanied by enhancements in local network connectivity in V1 microcircuits and increased dendritic complexity of V1 neurons. These effects were ameliorated by the acute application of GABAA receptor activators, which enhanced the activity of inhibitory neurons, or by reintroducing Fmr1 gene expression in knockout V1 neurons in both juvenile and young-adult mice. Overall, V1 plays an important role in the visual abnormalities of Fmr1KO mice and it could be possible to rescue the sensory disturbances in developed FXS and autism patients.

scholarly journals Impaired hippocampal representation of place in the Fmr1-knockout mouse model of Fragile X syndrome

2017 ◽  
Author(s):  
Tara Arbab ◽  
Cyriel MA Pennartz ◽  
Francesco P Battaglia
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Hippocampal Neurons ◽  
Spatial Information ◽  
Fragile X ◽  
Memory Function ◽  
Autism Spectrum ◽  
Spatial Representations ◽  
Potential Biomarker

AbstractFragile X syndrome (FXS) is an X-chromosome linked intellectual disability and the most common genetic 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 the stability of these spatial representations can be taken as an index of memory function. 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.

scholarly journals Impaired Dendritic Expression and Plasticity of h-Channels in the fmr1−/y Mouse Model of Fragile X Syndrome

Cell Reports ◽  
2012 ◽  
Vol 1 (3) ◽  
pp. 225-233 ◽  
Author(s):  
Darrin H. Brager ◽  
Arvin R. Akhavan ◽  
Daniel Johnston
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
H Channels

Mouse model of fragile X syndrome: Behavioral and hormonal response to stressors.

2009 ◽  
Vol 123 (3) ◽  
pp. 677-686 ◽  
Author(s):  
Darci M. Nielsen ◽  
Jeffrey J. Evans ◽  
William J. Derber ◽  
Kenzie A. Johnston ◽  
Mark L. Laudenslager ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Hormonal Response

scholarly journals The PDE10A Inhibitor TAK-063 Reverses Sound-Evoked EEG Abnormalities in a Mouse Model of Fragile X Syndrome

2021 ◽  
Author(s):  
Carrie R. Jonak ◽  
Manbir S. Sandhu ◽  
Samantha A. Assad ◽  
Jacqueline A. Barbosa ◽  
Mahindra Makhija ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X ◽  
Eeg Abnormalities

Aberrant differentiation of glutamatergic cells in neocortex of mouse model for fragile X syndrome

2009 ◽  
Vol 33 (2) ◽  
pp. 250-259 ◽  
Author(s):  
Topi A. Tervonen ◽  
Verna Louhivuori ◽  
Xiaohong Sun ◽  
Marie-Estelle Hokkanen ◽  
Claudius F. Kratochwil ◽  
...  
Keyword(s):  
Mouse Model ◽  
Fragile X Syndrome ◽  
Fragile X
Sign in / Sign up

Export Citation Format

Share Document