Fragile X Mental Retardation Protein and the Ribosome [PDF]
In this issue of Molecular Cell, Chen et al. (2014) provide evidence that FMRP represses translation by binding the ribosome, suggesting a novel form of translational control.
Yuriko Harigaya+3 more
openaire +3 more sources
On BC1 RNA and the fragile X mental retardation protein [PDF]
The fragile X mental retardation protein (FMRP), the functional absence of which causes fragile X syndrome, is an RNA-binding protein that has been implicated in the regulation of local protein synthesis at the synapse. The mechanism of FMRP's interaction with its target mRNAs, however, has remained controversial. In one model, it has been
Anna Iacoangeli+9 more
openaire +8 more sources
Fragile X Mental Retardation Syndrome: Structure of the KH1-KH2 Domains of Fragile X Mental Retardation Protein [PDF]
Fragile X syndrome is the most common form of inherited mental retardation in humans, with an estimated prevalence of about 1 in 4000 males. Although several observations indicate that the absence of functional Fragile X Mental Retardation Protein (FMRP) is the underlying basis of Fragile X syndrome, the structure and function of FMRP are currently ...
Irina Pozdnyakova+4 more
openaire +3 more sources
Fragile X mental retardation protein and synaptic plasticity [PDF]
Abstract Loss of the translational repressor FMRP causes Fragile X syndrome. In healthy neurons, FMRP modulates the local translation of numerous synaptic proteins. Synthesis of these proteins is required for the maintenance and regulation of long-lasting changes in synaptic strength.
Sidorov, Michael Samuel+2 more
openaire +4 more sources
The microRNA pathway and fragile X mental retardation protein [PDF]
Fragile X syndrome, one of the most common forms of inherited mental retardation, is caused by the functional loss of fragile X mental retardation protein (FMRP). MicroRNAs (miRNAs), a newly discovered class of small noncoding RNAs, have been implicated in multiple biological processes through posttranscriptional gene regulation.
Li Lin, Yujing Li, Peng Jin
openaire +3 more sources
Novel Features of dFMR1, the Drosophila Orthologue of the Fragile X Mental Retardation Protein [PDF]
FMRP belongs to a family of widely expressed proteins that contain RNA-binding domains. Although lack of human FMRP results in mental retardation, correlated with subtle synaptic changes, the precise role of FMRP remains elusive. The Drosophila genome contains a single gene homologous to the FXR family.
Annette Schenck+3 more
openalex +7 more sources
FMRP regulates GABAA receptor channel activity to control signal integration in hippocampal granule cells [PDF]
Fragile X syndrome, the most common inherited form of intellectual disability, is caused by loss of fragile X mental retardation protein (FMRP). GABAergic system dysfunction is one of the hallmarks of FXS, yet the underlying mechanisms remain poorly ...
Cavalli, Valeria+3 more
core +2 more sources
Molecular Insights into Mental Retardation: Multiple Functions for the Fragile X Mental Retardation Protein? [PDF]
Mental retardation is a frequent cause of intellectual and physical impairment. Several genes associated with mental retardation have been mapped to the X chromosome, among them, there is FMR1. The absence of or mutation in the Fragile Mental Retardation Protein, FMRP, is responsible for the Fragile X syndrome.
Zalfa, Francesca, Bagni, Claudia
openaire +2 more sources
Uncoupling of the endocannabinoid signalling complex in a mouse model of fragile X syndrome [PDF]
Fragile X syndrome, the most commonly known genetic cause of autism, is due to loss of the fragile X mental retardation protein, which regulates signal transduction at metabotropic glutamate receptor-5 in the brain.
DiPatrizio, Nicholas V.+12 more
core +5 more sources
Fragile X Mental Retardation Protein (FMRP) and the Spinal Sensory System [PDF]
The purpose of this chapter is to discuss the role of the fragile X mental retardation protein (FMRP) in the spinal sensory system and the potential for use of the mouse model of fragile X syndrome to better understand some aspects of the human syndrome as well as advance knowledge in other areas of investigation, such as pain amplification, an ...
Theodore J. Price+1 more
openalex +4 more sources