Literature DB >> 14994290

The fragile X syndrome: from molecular genetics to neurobiology.

Rob Willemsen1, Ben A Oostra, Gary J Bassell, Jason Dictenberg.   

Abstract

Since the identification of the FMR1 gene basic research has been focused on the molecular characterization of the FMR1 gene product, the fragile X mental retardation protein (FMRP). Recent developments in fragile X research have provided new insights and knowledge about the physiological function of FMRP in the cell and the nerve cell in particular. Currently, compelling evidence suggests a role for FMRP in the transport/translation of dendritically localized mRNAs. In addition, the identification of some of the target mRNAs of FMRP have led to an increased interest in the neurobiology of the syndrome. This review highlights the role of FMRP in dendritic mRNA transport/translation in relation to synaptic plasticity, a molecular mechanism implicated in learning and memory. Copyright 2004 Wiley-Liss, Inc.

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Year:  2004        PMID: 14994290     DOI: 10.1002/mrdd.20010

Source DB:  PubMed          Journal:  Ment Retard Dev Disabil Res Rev        ISSN: 1080-4013


  29 in total

Review 1.  Potential therapeutic interventions for fragile X syndrome.

Authors:  Josien Levenga; Femke M S de Vrij; Ben A Oostra; Rob Willemsen
Journal:  Trends Mol Med       Date:  2010-09-21       Impact factor: 11.951

2.  The fragile X mental retardation protein interacts with a distinct mRNA nuclear export factor NXF2.

Authors:  Dongmei Lai; Denny Sakkas; Yingqun Huang
Journal:  RNA       Date:  2006-06-21       Impact factor: 4.942

3.  Characterisation of Fmrp in zebrafish: evolutionary dynamics of the fmr1 gene.

Authors:  Sandra van 't Padje; Bart Engels; Lau Blonden; Lies-Anne Severijnen; Frans Verheijen; Ben A Oostra; Rob Willemsen
Journal:  Dev Genes Evol       Date:  2005-01-27       Impact factor: 0.900

4.  Modulation of dendritic spines and synaptic function by Rac1: a possible link to Fragile X syndrome pathology.

Authors:  Odelia Y N Bongmba; Luis A Martinez; Mary E Elhardt; Karlis Butler; Maria V Tejada-Simon
Journal:  Brain Res       Date:  2011-05-17       Impact factor: 3.252

Review 5.  FXTAS: a progressive neurologic syndrome associated with Fragile X premutation.

Authors:  Rob Willemsen; Edwin Mientjes; Ben A Oostra
Journal:  Curr Neurol Neurosci Rep       Date:  2005-09       Impact factor: 5.081

Review 6.  FMR1: a gene with three faces.

Authors:  Ben A Oostra; Rob Willemsen
Journal:  Biochim Biophys Acta       Date:  2009-02-21

Review 7.  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

8.  Normal protein composition of synapses in Ts65Dn mice: a mouse model of Down syndrome.

Authors:  Fabian Fernandez; Jonathan C Trinidad; Martina Blank; Dong-Dong Feng; Alma L Burlingame; Craig C Garner
Journal:  J Neurochem       Date:  2009-04-22       Impact factor: 5.372

9.  Murine hippocampal neurons expressing Fmr1 gene premutations show early developmental deficits and late degeneration.

Authors:  Yucui Chen; Flora Tassone; Robert F Berman; Paul J Hagerman; Randi J Hagerman; Rob Willemsen; Isaac N Pessah
Journal:  Hum Mol Genet       Date:  2010-01-01       Impact factor: 6.150

10.  Fragile X Mental Retardation Protein is Involved in Protein Synthesis-Dependent Collapse of Growth Cones Induced by Semaphorin-3A.

Authors:  Chanxia Li; Gary J Bassell; Yukio Sasaki
Journal:  Front Neural Circuits       Date:  2009-09-15       Impact factor: 3.492

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