Literature DB >> 12615009

Of mice and the fragile X syndrome.

R Frank Kooy1.   

Abstract

Fragile X syndrome is the most common cause of inherited mental retardation, and recently a number of mouse models have been generated to study the condition. Knockout of the gene associated with fragile X, Fmr1, results in mild, but consistent abnormalities, analogous to the clinical and pathological symptoms observed in human patients. Thus, many aspects of the syndrome can now be studied in mice, taking full advantage of the benefits of this model organism, including the short generation time and unlimited supply of tissue. The experimental data suggest that knockout of Fmr1 mildly disturbs a variety of processes in different brain regions.

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Year:  2003        PMID: 12615009     DOI: 10.1016/s0168-9525(03)00017-9

Source DB:  PubMed          Journal:  Trends Genet        ISSN: 0168-9525            Impact factor:   11.639


  30 in total

1.  Discrimination learning and attentional set formation in a mouse model of Fragile X.

Authors:  Kimberly S Casten; Annette C Gray; Rebecca D Burwell
Journal:  Behav Neurosci       Date:  2011-06       Impact factor: 1.912

2.  Epigenetic regulation of neuronal dendrite and dendritic spine development.

Authors:  Richard D Smrt; Xinyu Zhao
Journal:  Front Biol (Beijing)       Date:  2010-08

3.  Kinase pathway inhibition restores PSD95 induction in neurons lacking fragile X mental retardation protein.

Authors:  Ying Yang; Yang Geng; Dongyun Jiang; Lin Ning; Hyung Joon Kim; Noo Li Jeon; Anthony Lau; Lu Chen; Michael Z Lin
Journal:  Proc Natl Acad Sci U S A       Date:  2019-05-22       Impact factor: 11.205

4.  Deficits in the activity of presynaptic γ-aminobutyric acid type B receptors contribute to altered neuronal excitability in fragile X syndrome.

Authors:  Ji-Yong Kang; Jayashree Chadchankar; Thuy N Vien; Michelle I Mighdoll; Thomas M Hyde; Robert J Mather; Tarek Z Deeb; Menelas N Pangalos; Nicholas J Brandon; John Dunlop; Stephen J Moss
Journal:  J Biol Chem       Date:  2017-02-17       Impact factor: 5.157

5.  Fragile X mental retardation protein regulates synaptic and behavioral plasticity to repeated cocaine administration.

Authors:  Laura N Smith; Jakub P Jedynak; Miles R Fontenot; Carly F Hale; Karen C Dietz; Makoto Taniguchi; Feba S Thomas; Benjamin C Zirlin; Shari G Birnbaum; Kimberly M Huber; Mark J Thomas; Christopher W Cowan
Journal:  Neuron       Date:  2014-05-07       Impact factor: 17.173

6.  Fragile X and autism: Intertwined at the molecular level leading to targeted treatments.

Authors:  Randi Hagerman; Gry Hoem; Paul Hagerman
Journal:  Mol Autism       Date:  2010-09-21       Impact factor: 7.509

7.  Downregulation of tonic GABAergic inhibition in a mouse model of fragile X syndrome.

Authors:  Giulia Curia; Thomas Papouin; Philippe Séguéla; Massimo Avoli
Journal:  Cereb Cortex       Date:  2008-09-11       Impact factor: 5.357

8.  Absence of metabotropic glutamate receptor-mediated plasticity in the neocortex of fragile X mice.

Authors:  Brian M Wilson; Charles L Cox
Journal:  Proc Natl Acad Sci U S A       Date:  2007-02-07       Impact factor: 11.205

Review 9.  Potential pharmacological treatment of fragile X syndrome during adulthood.

Authors:  Zhao-Hui Xu; Ming-Gao Zhao
Journal:  Neurosci Bull       Date:  2009-10       Impact factor: 5.203

10.  A mouse model of the human Fragile X syndrome I304N mutation.

Authors:  Julie B Zang; Elena D Nosyreva; Corinne M Spencer; Lenora J Volk; Kiran Musunuru; Ru Zhong; Elizabeth F Stone; Lisa A Yuva-Paylor; Kimberly M Huber; Richard Paylor; Jennifer C Darnell; Robert B Darnell
Journal:  PLoS Genet       Date:  2009-12-11       Impact factor: 5.917

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