Literature DB >> 20298211

The developmental roles of FMRP.

Sally M Till1.   

Abstract

FXS (Fragile X syndrome) is the most common genetically inherited form of cognitive impairment. The predominant cause of the syndrome is the loss of a single protein, FMRP (Fragile X mental retardation protein). Many of the cognitive and behavioural features found in Fragile X individuals emerge during childhood and are associated with abnormal organization of cortical connections. However, although FMRP is expressed as early as embryogenesis, relatively little is known about its roles during development or how this may influence FXS phenotypes in adulthood. The present review focuses specifically on the evidence for the functions of FMRP during embryonic and early postnatal development. The current knowledge of the role of FMRP in FXS will be briefly summarized before addressing how alterations in the formation and refinement of neuronal connections and synaptic function that result from the loss of FMRP may in turn influence behaviours that are expressed during the first few postnatal weeks. I will then briefly highlight some outstanding questions about the developmental roles of FMRP and their possible relationship to symptoms found in adults with FXS.

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Year:  2010        PMID: 20298211     DOI: 10.1042/BST0380507

Source DB:  PubMed          Journal:  Biochem Soc Trans        ISSN: 0300-5127            Impact factor:   5.407


  17 in total

Review 1.  Coding region: the neglected post-transcriptional code.

Authors:  Eun Kyung Lee; Myriam Gorospe
Journal:  RNA Biol       Date:  2011-01-01       Impact factor: 4.652

2.  Aberrant frontal lobe maturation in adolescents with fragile X syndrome is related to delayed cognitive maturation.

Authors:  Signe Bray; Melissa Hirt; Booil Jo; Scott S Hall; Amy A Lightbody; Elizabeth Walter; Kelly Chen; Swetapadma Patnaik; Allan L Reiss
Journal:  Biol Psychiatry       Date:  2011-07-29       Impact factor: 13.382

3.  Cellular distribution of the fragile X mental retardation protein in the mouse brain.

Authors:  Diego A R Zorio; Christine M Jackson; Yong Liu; Edwin W Rubel; Yuan Wang
Journal:  J Comp Neurol       Date:  2016-09-16       Impact factor: 3.215

4.  Capturing the fragile X premutation phenotypes: a collaborative effort across multiple cohorts.

Authors:  Jessica Ezzell Hunter; Stephanie Sherman; Jim Grigsby; Cary Kogan; Kim Cornish
Journal:  Neuropsychology       Date:  2012-01-16       Impact factor: 3.295

5.  Constitutively active group I mGlu receptors and PKMzeta regulate synaptic transmission in developing perirhinal cortex.

Authors:  Isabella Panaccione; Rachel King; Gemma Molinaro; Barbara Riozzi; Giuseppe Battaglia; Ferdinando Nicoletti; Zafar I Bashir
Journal:  Neuropharmacology       Date:  2012-03-28       Impact factor: 5.250

6.  Fmr1 deficiency promotes age-dependent alterations in the cortical synaptic proteome.

Authors:  Bin Tang; Tingting Wang; Huida Wan; Li Han; Xiaoyan Qin; Yaoyang Zhang; Jian Wang; Chunlei Yu; Fulvia Berton; Walter Francesconi; John R Yates; Peter W Vanderklish; Lujian Liao
Journal:  Proc Natl Acad Sci U S A       Date:  2015-08-11       Impact factor: 11.205

7.  Genome-wide alteration of 5-hydroxymethylcytosine in a mouse model of fragile X-associated tremor/ataxia syndrome.

Authors:  Bing Yao; Li Lin; R Craig Street; Zachary A Zalewski; Jocelyn N Galloway; Hao Wu; David L Nelson; Peng Jin
Journal:  Hum Mol Genet       Date:  2013-10-09       Impact factor: 6.150

8.  Astroglial FMRP-dependent translational down-regulation of mGluR5 underlies glutamate transporter GLT1 dysregulation in the fragile X mouse.

Authors:  Haruki Higashimori; Lydie Morel; James Huth; Lothar Lindemann; Chris Dulla; Amaro Taylor; Mike Freeman; Yongjie Yang
Journal:  Hum Mol Genet       Date:  2013-02-07       Impact factor: 6.150

Review 9.  RNA-binding proteins, neural development and the addictions.

Authors:  C D Bryant; N Yazdani
Journal:  Genes Brain Behav       Date:  2016-01       Impact factor: 3.449

Review 10.  Dynamic duo - FMRP and TDP-43: Regulating common targets, causing different diseases.

Authors:  Diana Ferro; Stephen Yao; Daniela C Zarnescu
Journal:  Brain Res       Date:  2018-04-30       Impact factor: 3.252

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