The molecular biology of FMRP: new insights into fragile X syndrome

JD Richter, X Zhao - Nature Reviews Neuroscience, 2021 - nature.com
Fragile X mental retardation protein (FMRP) is the product of the fragile X mental retardation
1 gene (FMR1), a gene that—when epigenetically inactivated by a triplet nucleotide repeat …

Disruption of RNA metabolism in neurological diseases and emerging therapeutic interventions

JK Nussbacher, R Tabet, GW Yeo, C Lagier-Tourenne - Neuron, 2019 - cell.com
RNA binding proteins are critical to the maintenance of the transcriptome via controlled
regulation of RNA processing and transport. Alterations of these proteins impact multiple …

[HTML][HTML] FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism

JC Darnell, SJ Van Driesche, C Zhang, KYS Hung… - Cell, 2011 - cell.com
FMRP loss of function causes Fragile X syndrome (FXS) and autistic features. FMRP is a
polyribosome-associated neuronal RNA-binding protein, suggesting that it plays a key role …

Fragile X syndrome

RJ Hagerman, E Berry-Kravis, HC Hazlett… - Nature reviews Disease …, 2017 - nature.com
Fragile X syndrome (FXS) is the leading inherited form of intellectual disability and autism
spectrum disorder, and patients can present with severe behavioural alterations, including …

[HTML][HTML] Microarray identification of FMRP-associated brain mRNAs and altered mRNA translational profiles in fragile X syndrome

V Brown, P Jin, S Ceman, JC Darnell, WT O'Donnell… - Cell, 2001 - cell.com
Fragile X syndrome results from the absence of the RNA binding FMR protein. Here, mRNA
was coimmunoprecipitated with the FMRP ribonucleoprotein complex and used to …

Translational control in synaptic plasticity and cognitive dysfunction

SA Buffington, W Huang… - Annual review of …, 2014 - annualreviews.org
Activity-dependent changes in the strength of synaptic connections are fundamental to the
formation and maintenance of memory. The mechanisms underlying persistent changes in …

Fragile X mental retardation protein regulates translation by binding directly to the ribosome

E Chen, MR Sharma, X Shi, RK Agrawal, S Joseph - Molecular cell, 2014 - cell.com
Fragile X syndrome (FXS) is the most common form of inherited mental retardation, and it is
caused by loss of function of the fragile X mental retardation protein (FMRP). FMRP is an …

[HTML][HTML] The fragile X syndrome protein FMRP associates with BC1 RNA and regulates the translation of specific mRNAs at synapses

F Zalfa, M Giorgi, B Primerano, A Moro, A Di Penta… - Cell, 2003 - cell.com
The Fragile X syndrome, which results from the absence of functional FMRP protein, is the
most common heritable form of mental retardation. Here, we show that FMRP acts as a …

Drosophila fragile X-related gene regulates the MAP1B homolog Futsch to control synaptic structure and function

YQ Zhang, AM Bailey, HJG Matthies, RB Renden… - Cell, 2001 - cell.com
Fragile X mental retardation gene (FMR1) encodes an RNA binding protein that acts as a
negative translational regulator. We have developed a Drosophila fragile X syndrome model …

Fragile X mental retardation protein is translated near synapses in response to neurotransmitter activation

IJ Weiler, SA Irwin, AY Klintsova… - Proceedings of the …, 1997 - National Acad Sciences
Local translation of proteins in distal dendrites is thought to support synaptic structural
plasticity. We have previously shown that metabotropic glutamate receptor (mGluR1) …