Dysregulation and restoration of translational homeostasis in fragile X syndrome

Nature Reviews. Neuroscience
Joel D RichterE Klann

Abstract

Fragile X syndrome (FXS), the most-frequently inherited form of intellectual disability and the most-prevalent single-gene cause of autism, results from a lack of fragile X mental retardation protein (FMRP), an RNA-binding protein that acts, in most cases, to repress translation. Multiple pharmacological and genetic manipulations that target receptors, scaffolding proteins, kinases and translational control proteins can rescue neuronal morphology, synaptic function and behavioural phenotypes in FXS model mice, presumably by reducing excessive neuronal translation to normal levels. Such rescue strategies might also be explored in the future to identify the mRNAs that are critical for FXS pathophysiology.

References

Apr 12, 2001·Genes & Development·A C GingrasN Sonenberg
Oct 23, 2003·Human Molecular Genetics·Stephanie CemanStephen T Warren
Nov 15, 2003·Science·Jernej UleRobert B Darnell
Jun 29, 2004·Trends in Neurosciences·Mark F BearStephen T Warren
Aug 20, 2004·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Giovanni StefaniRobert B Darnell
Aug 27, 2004·Proceedings of the National Academy of Sciences of the United States of America·Edouard W KhandjianBarbara Bardoni
Oct 27, 2004·PLoS Biology·Bino JohnDebora S Marks
Nov 2, 2004·Nature Cell Biology·Peng JinStephen T Warren
Nov 19, 2004·Proceedings of the National Academy of Sciences of the United States of America·Ivan Jeanne WeilerWilliam T Greenough
Jan 7, 2005·Molecular & Cellular Proteomics : MCP·Yong Q ZhangKendal Broadie
Sep 2, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Shih-Chieh ChuangRobert K S Wong
Sep 30, 2005·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Raffaella GiuffridaMaria Vincenza Catania
Mar 15, 2006·Nature Chemical Biology·Marie-Eve BordeleauJerry Pelletier
May 18, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Ravi S MuddashettyGary J Bassell
Aug 19, 2007·Nucleic Acids Research·Lakshmi Menon, Mihaela-Rita Mihailescu
Dec 21, 2007·Neuron·Gül DölenMark F Bear
Dec 28, 2007·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Usha NarayananStephen T Warren
Jan 19, 2008·The Journal of Physiology·Gül Dölen, Mark F Bear
Mar 11, 2008·Nature Chemical Biology·Shuang ChangStephen T Warren
Aug 22, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·N Ruth ZearfossJoel D Richter
Oct 3, 2008·Proceedings of the National Academy of Sciences of the United States of America·Lujian LiaoJohn R Yates
Nov 4, 2008·Nature·Donny D LicatalosiRobert B Darnell
Nov 14, 2008·The Journal of Neuroscience : the Official Journal of the Society for Neuroscience·Xia-Lian XuFen-Biao Gao
Mar 17, 2009·Current Opinion in Cell Biology·Rachel Groppo, Joel D Richter

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Citations

Jul 28, 2016·Biological Psychiatry·Eunee LeeEunjoon Kim
Jun 19, 2016·Proceedings of the National Academy of Sciences of the United States of America·Aoife C McMahon, Michael Rosbash
Jun 1, 2016·Journal of Neurochemistry·Dominique Fernandes, Ana Luísa Carvalho
May 21, 2016·Genes, Brain, and Behavior·B ZupanM Toth
Aug 17, 2016·Trends in Biochemical Sciences·Clive R BramhamChristopher G Proud
Oct 30, 2016·Biochimie·Ricardos TabetHervé Moine
Sep 25, 2016·Proceedings of the National Academy of Sciences of the United States of America·Kirsty SawickaR Suzanne Zukin
Oct 16, 2016·The Journal of Physiology·Jörg Striessnig
Nov 8, 2016·Trends in Genetics : TIG·Rico SchieweckMichael A Kiebler
Dec 1, 2017·Acta Biochimica Et Biophysica Sinica·Risa Kashima, Akiko Hata
Mar 27, 2018·The European Journal of Neuroscience·John J FoxeMark T Wallace
Apr 4, 2018·Molecular and Cellular Biology·Philippe P Roux, Ivan Topisirovic
Jun 2, 2018·FEBS Letters·Christopher Gallagher, Andres Ramos
Jun 7, 2018·Journal of Neurochemistry·Jocelyn Widagdo, Victor Anggono
Oct 31, 2018·Proceedings of the National Academy of Sciences of the United States of America·Botao LiuJoel D Richter
Oct 27, 2018·Annual Review of Medicine·Ilse GantoisNahum Sonenberg
Feb 2, 2019·Translational Psychiatry·Eleonora RosinaClaudia Bagni
Sep 30, 2017·Nature Reviews. Disease Primers·Randi J HagermanPaul J Hagerman
Nov 27, 2019·Acta Neuropathologica·Yi TanThomas Koeglsperger
Mar 1, 2020·International Journal of Molecular Sciences·Han Kyoung Choe, Jun Cho
Jun 12, 2020·The FEBS Journal·Mohamed S TahaMohammad R Ahmadian
Sep 4, 2020·Critical Reviews in Biochemistry and Molecular Biology·Mrinmoyee MajumderViswanathan Palanisamy
Oct 2, 2020·Physiological Reviews·Rico SchieweckMichael A Kiebler
Dec 29, 2015·Neuropsychopharmacology : Official Publication of the American College of Neuropsychopharmacology·Aditi BhattacharyaEric Klann
Jun 5, 2016·G3 : Genes - Genomes - Genetics·Mala MisraElizabeth R Gavis
Jun 9, 2018·Proceedings of the National Academy of Sciences of the United States of America·Kendrick J JonesWeifeng Xu
Aug 29, 2018·Brain Plasticity·Natalie E PatzlaffXinyu Zhao
Jul 30, 2018·Molecular Neurobiology·Joanna J ChmielewskaMagdalena Dziembowska

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