FMRP stalls ribosomal translocation on mRNAs linked to synaptic function and autism
Jennifer C. Darnell,Sarah J. Van Driesche,Chaolin Zhang,Ka Ying Sharon Hung,Aldo Mele,Claire E. Fraser,Elizabeth F. Stone,Cynthia Chen,John J. Fak,Sung Wook Chi,Donny D. Licatalosi,Joel D. Richter,Robert B. Darnell,Robert B. Darnell +13 more
TLDR
A brain polyribosome-programmed translation system is developed, revealing that FMRP reversibly stalls ribosomes specifically on its target mRNAs and suggests multiple targets for clinical intervention in FXS and ASD.About:
This article is published in Cell.The article was published on 2011-07-22 and is currently open access. It has received 1861 citations till now. The article focuses on the topics: FMR1 & RNA-binding protein.read more
Citations
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Journal ArticleDOI
FMRP-dependent Mdm2 dephosphorylation is required for MEF2-induced synapse elimination.
TL;DR: Detailed mechanisms of synapse elimination in health and a developmental brain disorder are revealed and expression of a dephosphomimetic of Mdm2 rescues PSD-95 ubiquitination, degradation and synapses elimination in Fmr1 KO neurons.
Posted ContentDOI
FUS-ALS mutants alter FMRP phase separation equilibrium and impair protein translation
Nicol Birsa,Nicol Birsa,Agnieszka M. Ule,Maria Giovanna Garone,Brian Tsang,Francesca Mattedi,P.A. Chong,Jack Humphrey,Seth Jarvis,M. Pisiren,Oscar G. Wilkins,Oscar G. Wilkins,Michael L Nosella,Anny Devoy,Cristian Bodo,R. Fernandez de la Fuente,Elizabeth M. C. Fisher,Alessandro Rosa,Gabriella Viero,Julie D. Forman-Kay,Giampietro Schiavo,Giampietro Schiavo,Pietro Fratta +22 more
TL;DR: It is found that mutant FUS forms cytoplasmic condensates that promote the phase separation of FMRP, another RBP associated with neurodegeneration and robustly involved in translation regulation.
Journal ArticleDOI
mTOR Inhibitors in Children: Current Indications and Future Directions in Neurology
Anna Jeong,Michael Wong +1 more
TL;DR: The approved indications for mTOR inhibitor in TSC, the potential future applications of mTOR inhibitors in T SC and other neurological conditions, and the safety considerations applicable to mTOR therapy in the pediatric population are discussed.
Journal ArticleDOI
Altered Developmental Expression of the Astrocyte-Secreted Factors Hevin and SPARC in the Fragile X Mouse Model.
TL;DR: Hevin and SPARC showed altered expression patterns in Fmr1 KO mice compared to WT, in a brain-region specific manner, which imply aberrant astrocyte signaling in FXS and suggest that the altered expression of hevin andSPARC contributes to abnormal synaptic development inFXS.
Book ChapterDOI
Axon-TRAP-RiboTag: Affinity Purification of Translated mRNAs from Neuronal Axons in Mouse In Vivo.
TL;DR: Axon-TRAP-RiboTag is developed, a TRAP-based method that allows purification and identification of translated mRNAs from distal neuronal axons in mouse, and it is identified more than 2000 of translatedmRNAs in retinal ganglion cell (RGC) axONS in vivo.
References
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TL;DR: In this paper, the authors present a model for the analysis of variance in a single-classification and two-way and multiway analysis of Variance with the assumption of correlation.
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