| Publication |
Sentence |
Publish Date |
Extraction Date |
Species |
| Bumwhee Lee, Laura Beuhler, Hye Young Le. The Primary Ciliary Deficits in Cerebellar Bergmann Glia of the Mouse Model of Fragile X Syndrome. Cerebellum (London, England). 2022-04-19. PMID:35438410. |
we further found reduced granule neuron precursor (gnp) proliferation and thickness of the external germinal layer (egl) in fmr1 ko mice, implicating that primary ciliary deficits in bergmann glia may contribute to cerebellar developmental phenotypes in fxs, as shh signaling through primary cilia in bergmann glia is known to mediate proper gnp proliferation in the egl. |
2022-04-19 |
2023-08-13 |
mouse |
| Shivaprasad H Sathyanarayana, Jasmine A Saunders, Jacob Slaughter, Kamran Tariq, Rajarshi Chakrabarti, Madhumala K Sadanandappa, Bryan W Luikart, Giovanni Bosc. Proceedings of the National Academy of Sciences of the United States of America. vol 119. issue 15. 2022-04-18. PMID:35394871. |
to understand the interaction of pten and fmrp, we investigated the dentate gyrus granule neuron development in pten and fmr1 knockout (ko) mice. |
2022-04-18 |
2023-08-16 |
mouse |
| Yeri J Song, Bo Xing, Aaron J Barbour, Chengwen Zhou, Frances E Jense. Dysregulation of GABAA Receptor-Mediated Neurotransmission during the Auditory Cortex Critical Period in the Fragile X Syndrome Mouse Model. Cerebral cortex (New York, N.Y. : 1991). vol 32. issue 1. 2021-12-08. PMID:34223875. |
here, we examined gabaa receptor (gabaar)-mediated inhibitory synaptic transmission within the auditory cortex, characterizing normal intracortical circuit development patterns in wild-type (wt) mice and examining their dysregulation in developing fmr1 knock-out (ko) mice. |
2021-12-08 |
2023-08-13 |
mouse |
| Yeri J Song, Bo Xing, Aaron J Barbour, Chengwen Zhou, Frances E Jense. Dysregulation of GABAA Receptor-Mediated Neurotransmission during the Auditory Cortex Critical Period in the Fragile X Syndrome Mouse Model. Cerebral cortex (New York, N.Y. : 1991). vol 32. issue 1. 2021-12-08. PMID:34223875. |
specifically, fmr1 ko l2/3 pyramidal neurons have enhanced developmental sensitivity to pharmacological gabaar modulators, altered maturation of gabaar voltage-dependent conductance, with additional presynaptic gaba release alterations. |
2021-12-08 |
2023-08-13 |
mouse |
| Benjamin D Auerbach, Senthilvelan Manohar, Kelly Radziwon, Richard Salv. Auditory hypersensitivity and processing deficits in a rat model of fragile X syndrome. Neurobiology of disease. vol 161. 2021-12-06. PMID:34751141. |
finally, we demonstrated that antagonism of metabotropic glutamate receptor 5 (mglu5) selectively and dose-dependently restored normal loudness growth in fmr1 ko rats, suggesting a pharmacologic approach for alleviating sensory hypersensitivity associated with fx. |
2021-12-06 |
2023-08-13 |
rat |
| Rohini Subrahmanyam, Deepanjali Dwivedi, Zubin Rashid, Katherine Bonnycastle, Michael A Cousin, Sumantra Chattarj. Reciprocal regulation of spontaneous synaptic vesicle fusion by Fragile X mental retardation protein and group I metabotropic glutamate receptors. Journal of neurochemistry. vol 158. issue 5. 2021-11-17. PMID:34327719. |
here we combined electrophysiological and fluorescence imaging analyses on primary hippocampal cultures prepared from an fmr1 knockout (ko) rat model. |
2021-11-17 |
2023-08-13 |
rat |
| Hayes Wong, Alexander W M Hooper, Yosuke Niibori, Shiron J Lee, Luca A Hategan, Liang Zhang, Subha Karumuthil-Melethil, Sally M Till, Peter C Kind, Olivier Danos, Joseph T Bruder, David R Hampso. Sexually dimorphic patterns in electroencephalography power spectrum and autism-related behaviors in a rat model of fragile X syndrome. Neurobiology of disease. vol 146. 2021-10-25. PMID:33031903. |
eeg analysis demonstrated that compared to wild-type, male fmr1 ko rats showed an increase in gamma frequency band power in the frontal cortex during the sleep-like immobile state, and both male and female ko rats failed to show an increase in delta frequency power in the sleep-like state, as observed in wild-type rats. |
2021-10-25 |
2023-08-13 |
human |
| Hayes Wong, Alexander W M Hooper, Yosuke Niibori, Shiron J Lee, Luca A Hategan, Liang Zhang, Subha Karumuthil-Melethil, Sally M Till, Peter C Kind, Olivier Danos, Joseph T Bruder, David R Hampso. Sexually dimorphic patterns in electroencephalography power spectrum and autism-related behaviors in a rat model of fragile X syndrome. Neurobiology of disease. vol 146. 2021-10-25. PMID:33031903. |
our findings highlight sex differences between male and female fmr1 ko rats, and indicate that the rat model of fxs could be a useful tool for the development of new therapeutics for treating this debilitating neurodevelopmental disorder. |
2021-10-25 |
2023-08-13 |
human |
| Jenny L Fulks, Bliss E O'Bryhim, Sara K Wenzel, Stephen C Fowler, Elena Vorontsova, Jonathan W Pinkston, Andrea N Ortiz, Michael A Johnso. Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome. ACS chemical neuroscience. vol 1. issue 10. 2021-10-20. PMID:21116467. |
in this study we evaluated the relationship between amphetamine-induced behavioral alterations and dopamine release and uptake characteristics in fmr1 knockout (fmr1 ko) mice, which model fragile x syndrome. |
2021-10-20 |
2023-08-12 |
mouse |
| Jenny L Fulks, Bliss E O'Bryhim, Sara K Wenzel, Stephen C Fowler, Elena Vorontsova, Jonathan W Pinkston, Andrea N Ortiz, Michael A Johnso. Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome. ACS chemical neuroscience. vol 1. issue 10. 2021-10-20. PMID:21116467. |
however, at 15 (the age of behavioral testing) and 20 weeks of age, dopamine per pulse and the maximum rate of dopamine uptake was diminished in fmr1 ko mice compared to wt mice. |
2021-10-20 |
2023-08-12 |
mouse |
| Jenny L Fulks, Bliss E O'Bryhim, Sara K Wenzel, Stephen C Fowler, Elena Vorontsova, Jonathan W Pinkston, Andrea N Ortiz, Michael A Johnso. Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome. ACS chemical neuroscience. vol 1. issue 10. 2021-10-20. PMID:21116467. |
however, dopamine release, uncorrected for uptake and normalized against the corresponding pre-drug release peaks, increased in fmr1 ko mice, but not in wt mice. |
2021-10-20 |
2023-08-12 |
mouse |
| Jenny L Fulks, Bliss E O'Bryhim, Sara K Wenzel, Stephen C Fowler, Elena Vorontsova, Jonathan W Pinkston, Andrea N Ortiz, Michael A Johnso. Dopamine Release and Uptake Impairments and Behavioral Alterations Observed in Mice that Model Fragile X Mental Retardation Syndrome. ACS chemical neuroscience. vol 1. issue 10. 2021-10-20. PMID:21116467. |
collectively, these data are consistent with a scenario in which a decrease in extracellular dopamine levels in the striatum result in diminished expression of focused stereotypy in fmr1 ko mice. |
2021-10-20 |
2023-08-12 |
mouse |
| Dai-Chi Liu, Kwan Young Lee, Simon Lizarazo, Jessie K Cook, Nien-Pei Tsa. ER stress-induced modulation of neural activity and seizure susceptibility is impaired in a fragile X syndrome mouse model. Neurobiology of disease. vol 158. 2021-10-14. PMID:34303799. |
imbalanced neuronal excitability homeostasis is commonly observed in patients with fragile x syndrome (fxs) and the animal model of fxs, the fmr1 ko. |
2021-10-14 |
2023-08-13 |
mouse |
| Karo Talvio, Katja M Kanninen, Anthony R White, Jari Koistinaho, Maija L Castré. Increased iron content in the heart of the Fmr1 knockout mouse. Biometals : an international journal on the role of metal ions in biology, biochemistry, and medicine. vol 34. issue 4. 2021-10-11. PMID:34089433. |
we assessed the concentrations of 23 trace minerals in different tissues (brain, spleen, heart and liver) of fmr1 knockout (ko) mice that display the main phenotype of fragile x syndrome (fxs), an intellectual disability syndrome and the best-known monogenic model of autism spectrum disorder (asd). |
2021-10-11 |
2023-08-13 |
mouse |
| Jason Arsenault, Alexander W M Hooper, Shervin Gholizadeh, Tian Kong, Laura K Pacey, Enea Koxhioni, Yosuke Niibori, James H Eubanks, Lu-Yang Wang, David R Hampso. Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex. Human molecular genetics. vol 29. issue 23. 2021-09-28. PMID:33084871. |
here, we report a homeostatic relationship between these two key regulators of gene expression in mouse models of fxs (fmr1 knockout (ko)) and rett syndrome (mecp2 ko). |
2021-09-28 |
2023-08-13 |
mouse |
| Jason Arsenault, Alexander W M Hooper, Shervin Gholizadeh, Tian Kong, Laura K Pacey, Enea Koxhioni, Yosuke Niibori, James H Eubanks, Lu-Yang Wang, David R Hampso. Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex. Human molecular genetics. vol 29. issue 23. 2021-09-28. PMID:33084871. |
we found that the level of mecp2 protein in the cerebral cortex was elevated in fmr1 ko mice, whereas mecp2 ko mice displayed reduced levels of fmrp, implicating interplay between the activities of mecp2 and fmrp. |
2021-09-28 |
2023-08-13 |
mouse |
| Jason Arsenault, Alexander W M Hooper, Shervin Gholizadeh, Tian Kong, Laura K Pacey, Enea Koxhioni, Yosuke Niibori, James H Eubanks, Lu-Yang Wang, David R Hampso. Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex. Human molecular genetics. vol 29. issue 23. 2021-09-28. PMID:33084871. |
additionally, the level of mecp2 in the posterior cortex correlated with the severity of the hyperactive phenotype in fmr1 ko mice. |
2021-09-28 |
2023-08-13 |
mouse |
| Jason Arsenault, Alexander W M Hooper, Shervin Gholizadeh, Tian Kong, Laura K Pacey, Enea Koxhioni, Yosuke Niibori, James H Eubanks, Lu-Yang Wang, David R Hampso. Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex. Human molecular genetics. vol 29. issue 23. 2021-09-28. PMID:33084871. |
our findings also suggest that mecp2 overexpression in fmr1 ko mouse posterior cerebral cortex may contribute to the fragile x locomotor hyperactivity phenotype. |
2021-09-28 |
2023-08-13 |
mouse |
| Cristine R Casingal, Takako Kikkawa, Hitoshi Inada, Yukio Sasaki, Noriko Osum. Identification of FMRP target mRNAs in the developmental brain: FMRP might coordinate Ras/MAPK, Wnt/β-catenin, and mTOR signaling during corticogenesis. Molecular brain. vol 13. issue 1. 2021-09-03. PMID:33323119. |
we indeed showed overactivation of mtor signaling via an increase in mtor phosphorylation in the fmr1 knockout (fmr1 ko) neocortex. |
2021-09-03 |
2023-08-13 |
mouse |
| Jessica L Huebschman, Megan C Davis, Catherina Tovar Pensa, Yuhong Guo, Laura N Smit. The fragile X mental retardation protein promotes adjustments in cocaine self-administration that preserve reinforcement level. The European journal of neuroscience. vol 54. issue 3. 2021-08-20. PMID:34133054. |
utilizing a cocaine intravenous self-administration (ivsa) assay in fmr1 knockout (ko) and wild-type (wt) littermate mice, we find that, despite normal acquisition and extinction learning, fmr1 ko mice fail to make a normal upward shift in responding during dose-response testing. |
2021-08-20 |
2023-08-13 |
mouse |