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Showing 1 to 20 of 32 for “"Fragile X Mental Retardation Protein"”.
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Fragile X Mental Retardation Protein Induces Synapse Loss Through Acute Postsynaptic Translational Regulation
Fragile X Syndrome (FXS) is the most common form of inherited mental retardation. The root cause of FXS is loss of the function of a single protein: the Fragile X Mental Retardation Protein (FMRP). FMRP is an RNA-binding protein that plays a complex role in translational regulation. FMRP may be an …
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Elucidating the Role of the Fragile-X Mental Retardation Protein in the Central Nervous System
The discovery that proteins are synthesized at synapses, away from somata where most proteins are made, is fairly recent, and only a couple of proteins are currently known to be made near synapses; none have been shown to be made in response to changes in the synaptic environment. Proteins made in …
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Mechanism of Translational Control by the Fragile X Mental Retardation Protein and Creation of the FMRP CTAG Mouse
<p>The Fragile X Mental Retardation Protein (FMRP) is a neuronal RNA-binding protein that is predominantly associated with polyribosomes. Loss of FMRP results in Fragile X Syndrome, characterized by mental retardation, autism and epilepsy. FMRP was recently found to be associated with a specific …
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A More General Role for the Fragile X Mental Retardation Protein in Pruning of Both Synaptic and Dendritic Processes
… does appear to be necessary for normal developmental dendritic retraction in both somatosensory whisker barrel cortex and olfactory bulb of FraX mice. Further analysis of both normal development and anatomical and metabolic activity in somatosensory whisker barrel cortex using nissl and …
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Deletion of FMR1 results in sex-specific changes in behavior.
Fragile X Syndrome (FXS) is a neurodevelopmental disorder caused by excessive trinucleotide (CGG) repeats in the FMR1 gene coding for fragile x mental retardation protein (FMRP). In humans, this disorder is characterized by intellectual disability, as well as other behavioral abnormalities, such as …
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The Role of G-Quadruplex RNA Motif in Fragile X Syndrome
Fragile X syndrome (FXS), the most common cause of inherited mental impairment, is caused by the loss of expression of the fragile X mental retardation protein (FMRP). As an RNA binding protein, FMRP has been proposed to regulate the transport and translation of specific message RNA (mRNA). It has …
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Kh Domains on Brain Polyribosomes: FMRP and Nova in Translational Regulation
<p>The regulation of protein synthesis is an important aspect of the control of gene expression in neurons and is thought to contribute to neurologic diseases such as Fragile X mental retardation syndrome. We demonstrate that several neuronal RNA-binding proteins implicated in human disease are …
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Polarizable Simulations of the bcl-2 DNA G-Quadruplex and FMRP RNA G-Quadruplex:Duplex Junction Binding Protein
… the second is the sc1 RNA GQ, which binds to the Fragile-X Mental Retardation Protein (FMRP) and is implicated in the development of Fragile X Syndrome (FXS). Aberrant bcl-2 GQ conformations result in increased production of the BCL2 protein, which is an apoptosis inhibitor. As such, we aim to …
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Dysregulated ERK signal pathway and immune profiles in Fragile X Syndrome
Fragile X Syndrome (FXS) is the leading cause of inherited mental retardation, and the most common identified genetic cause of autism. Lack of production of the Fragile X Mental Retardation Protein (FMRP) leads to changes in dendritic morphology and resultant cognitive and behavioral manifestations …
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Ultrastructure and morphometric analysis of hippocampal synapses in the Fmr1-/y mouse model of fragile X syndrome
Fragile X Syndrome (FXS) is a prevalent monogenic disease, often presenting with cognitive and neurological disorders including autism and epilepsy. The Fmr1 gene - transcriptionally silenced in FXS - normally encodes the Fragile X Mental Retardation Protein (FMRP), which acts as an activity …
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A Study on an FMRP-Mediated Translational Switch in the MGluR-Triggered Translation of Arc and Synaptic Plasticity
… glutamate receptor (mGluR)-stimulated protein synthesis and long-term synaptic depression (mGluR-LTD) are altered in a mouse model of Fragile X Syndrome, Fmr1 knockout (KO) mouse. Fmr1 encodes the Fragile X mental retardation protein (FMRP), a dendritic RNA-binding protein that …
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Novel Functions for Neuronal RNA Processing Bodies in the Control of Axon Terminal Growth in Drosophila Melanogaster
… HPat interacts with the <em>Drosophila</em> Fragile X Mental Retardation Protein (dFMR1), to regulate neuronal structure in a <em>Drosophila melanogaster</em> fragile X model. First, we demonstrated that HPat interacts biochemically with dFMRP in an RNAse independent manner. Second, we show …
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An Examination of the Mechanisms of Neocortical Network Excitability in a Mouse Model of Fragile X Syndrome
Fragile X Syndrome (FXS) is the most common heritable form of mental retardation. FXS is caused by loss of function mutations in the product of the Fmr1 gene, the Fragile X Mental Retardation Protein (FMRP). Many FXS patients display symptoms that are indicative of hyperexcitable circuitry, …
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Metabotropic glutamate receptor 5 : a therapeutic target in Fragile X and a regulator of plasticity in visual cortex
The synaptic proteins Fragile X mental retardation protein (FMRP) and metabotropic glutamate receptor 5 (mGluR5) act in functional opposition to regulate local translation of synaptic mRNAs. Fragile X is the most common form of inherited intellectual disability and autism, and is expressed by a …
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Identification of Loss of Specific FMRP-RNA Interactions as a Cause of Fragile X Syndrome
<p>Fragile X Syndrome presents with a clinical picture of moderate to severe mental retardation and behavioral abnormalities including autistic features resulting from the loss of function of a RNA-binding protein, Fragile X mental retardation protein (FMRP). This work is devoted to the …
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Identification and Characterization of Novel Mechanisms of Functional and Structural Synapse Remodeling: Focus on Vav Guanine Nucleotide Exchange Factors and MEF2 Transcription Factors
… strength. Investigation of numerous neurodevelopmental and psychiatric disorders reveals dysfunctions in synapse formation and function; however, underlying molecular mechanisms remain poorly understood. In Part One of this study, I identify a novel role for Vav guanine nucleotide exchange …
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PART I CRYSTAL STRUCTURE OF A DIMERIZATION DOMAIN OF DROSOPHILA CAPRIN. PART II CHARACTERIZATION OF TWO CAS13B CRISPR-CAS SYSTEMS FROM PORPHYROMONAS GINGIVALIS
… shares conversed HR1 domain with Caprin protein family members, which are RNA binding proteins that play critical roles in many important biological processes, such as synaptic plasticity, stress response, innate immune response and cellular proliferation. One of the Caprin protein family …
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Modelling fragile X syndrome in rats: new directions in translational research
Fragile X syndrome (FXS) is the leading single gene cause of intellectual disability and Autism Spectrum Disorder (ASD). It is caused by epigenetic silencing of the fragile X mental retardation gene (FMR1), causing a loss of Fragile-X Mental Retardation Protein (FMRP). Over the last 2 decades, much …
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Analysis of the expression and effects of vascular endothelial growth factor family of molecules on Fragile X Syndrome abnormalities in a mouse model
Fragile X Syndrome (FXS) is the most common form of inherited mental retardation affecting 1:3600 males and 1:8000 females (Cornish et al., 2008). The primary cause is a silencing of the FMR1 gene, via increased CGG trinucleotide repeats, which encodes for the Fragile X Mental Retardation Protein …
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Characterization and Modulation of the Emergent Material Properties of Biomolecular Condensates
… biology and our understanding of cell compartmentalization. These organelles lack a distinct phospholipid bilayer and interact with the cytoplasm mediated by a liquid-liquid interface. The emergent properties and characterization of these organelles are essential for decoding the mechanisms …
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