A novel role of fragile X mental retardation protein in pre-mRNA alternative splicing through RNA-binding protein 14

Neuroscience ◽  
2017 ◽  
Vol 349 ◽  
pp. 64-75 ◽  
Author(s):  
Lin-Tao Zhou ◽  
Shun-Hua Ye ◽  
Hai-Xuan Yang ◽  
Yong-Ting Zhou ◽  
Qi-Hua Zhao ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Alternative Splicing ◽  
Rna Binding ◽  
Fragile X ◽  
Binding Protein ◽  
Rna Binding Protein ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein

scholarly journals The RNA binding protein fragile X mental retardation protein promotes myelin sheath growth

Glia ◽  
2019 ◽  
Vol 68 (3) ◽  
pp. 495-508 ◽  
Author(s):  
Caleb A. Doll ◽  
Katie M. Yergert ◽  
Bruce H. Appel
Keyword(s):  
Mental Retardation ◽  
Myelin Sheath ◽  
Rna Binding ◽  
Fragile X ◽  
Binding Protein ◽  
Rna Binding Protein ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein

scholarly journals The nuclear MicroSpherule protein 58 is a novel RNA-binding protein that interacts with fragile X mental retardation protein in polyribosomal mRNPs from neurons

2006 ◽  
Vol 15 (9) ◽  
pp. 1525-1538 ◽  
Author(s):  
Laetitia Davidovic ◽  
Elias Bechara ◽  
Maud Gravel ◽  
Xavier H. Jaglin ◽  
Sandra Tremblay ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Rna Binding ◽  
Fragile X ◽  
Binding Protein ◽  
Rna Binding Protein ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein

Molecular Functions of the Mammalian Fragile X Mental Retardation Protein: Insights Into Mental Retardation and Synaptic Plasticity

2013 ◽  
Author(s):  
Claudia Bagni ◽  
Eric Klann
Keyword(s):  
Mental Retardation ◽  
Binding Proteins ◽  
Rna Binding ◽  
Rna Binding Proteins ◽  
Fragile X ◽  
Rna Binding Protein ◽  
Fragile X Mental Retardation ◽  
The Family ◽  
Mental Retardation Protein ◽  
The Brain

Chapter 8 discusses how Fragile X syndrome (FXS) is caused by the absence of the RNA-binding protein fragile X mental retardation protein (FMRP). FMRP is highly expressed in the brain and gonads, the two organs mainly affected in patients with the syndrome. Functionally, FMRP belongs to the family of RNA-binding proteins, shuttling from the nucleus to the cytoplasm, and, as shown for other RNA-binding proteins, forms large messenger ribonucleoparticles.

scholarly journals The RNA-binding fragile-X mental retardation protein and its role beyond the brain

2020 ◽  
Vol 12 (4) ◽  
pp. 903-916 ◽  
Author(s):  
Cassandra Malecki ◽  
Brett D. Hambly ◽  
Richmond W. Jeremy ◽  
Elizabeth N. Robertson
Keyword(s):  
Mental Retardation ◽  
Rna Binding ◽  
Fragile X ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein ◽  
The Brain

scholarly journals Role of microRNA Pathway in Mental Retardation

2007 ◽  
Vol 7 ◽  
pp. 146-154 ◽  
Author(s):  
Abrar Qurashi ◽  
Shuang Chang ◽  
Peng Jin
Keyword(s):  
Mental Retardation ◽  
Fragile X Syndrome ◽  
Genetic Basis ◽  
Fragile X ◽  
Biological Pathways ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein ◽  
Mirna Pathway ◽  
Microrna Pathway

Deficits in cognitive functions lead to mental retardation (MR). Understanding the genetic basis of inherited MR has provided insights into the pathogenesis of MR. Fragile X syndrome is one of the most common forms of inherited MR, caused by the loss of functional Fragile X Mental Retardation Protein (FMRP).MicroRNAs (miRNAs) are endogenous, single-stranded RNAs between 18 and 25 nucleotides in length, which have been implicated in diversified biological pathways. Recent studies have linked the miRNA pathway to fragile X syndrome. Here we review the role of the miRNA pathway in fragile X syndrome and discuss its implication in MR in general.

scholarly journals Kinase pathway inhibition restores PSD95 induction in neurons lacking fragile X mental retardation protein

2019 ◽  
pp. 201812056
Author(s):  
Ying Yang ◽  
Yang Geng ◽  
Dongyun Jiang ◽  
Lin Ning ◽  
Hyung Joon Kim ◽  
...  
Keyword(s):  
Protein Synthesis ◽  
Mental Retardation ◽  
Rna Binding ◽  
Fragile X ◽  
Alternative Pathways ◽  
Fragile X Mental Retardation ◽  
Mrna Targets ◽  
Mental Retardation Protein ◽  
Pathway Inhibition

Fragile X syndrome (FXS) is the leading monogenic cause of autism and intellectual disability. FXS is caused by loss of expression of fragile X mental retardation protein (FMRP), an RNA-binding protein that regulates translation of numerous mRNA targets, some of which are present at synapses. While protein synthesis deficits have long been postulated as an etiology of FXS, how FMRP loss affects distributions of newly synthesized proteins is unknown. Here we investigated the role of FMRP in regulating expression of new copies of the synaptic protein PSD95 in an in vitro model of synaptic plasticity. We find that local BDNF application promotes persistent accumulation of new PSD95 at stimulated synapses and dendrites of cultured neurons, and that this accumulation is absent in FMRP-deficient mouse neurons. New PSD95 accumulation at sites of BDNF stimulation does not require known mechanisms regulating FMRP–mRNA interactions but instead requires the PI3K-mTORC1-S6K1 pathway. Surprisingly, in FMRP-deficient neurons, BDNF induction of new PSD95 accumulation can be restored by mTORC1-S6K1 blockade, suggesting that constitutively high mTORC1-S6K1 activity occludes PSD95 regulation by BDNF and that alternative pathways exist to mediate induction when mTORC1-S6K1 is inhibited. This study provides direct evidence for deficits in local protein synthesis and accumulation of newly synthesized protein in response to local stimulation in FXS, and supports mTORC1-S6K1 pathway inhibition as a potential therapeutic approach for FXS.

scholarly journals Decreased Nociceptive Sensitization in Mice Lacking the Fragile X Mental Retardation Protein: Role of mGluR1/5 and mTOR

2007 ◽  
Vol 27 (51) ◽  
pp. 13958-13967 ◽  
Author(s):  
T. J. Price ◽  
M. H. Rashid ◽  
M. Millecamps ◽  
R. Sanoja ◽  
J. M. Entrena ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Fragile X ◽  
Nociceptive Sensitization ◽  
Fragile X Mental Retardation ◽  
Mental Retardation Protein
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