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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Both maintenance and avoidance of RNA-binding protein interactions constrain coding sequence evolution
TL;DR: The challenge faced by the CDSs is not only one of attracting the right RBPs but also of avoiding the wrong ones, all while also evolving under selection pressures related to protein structure.
Journal ArticleDOI
A Drosophila model of Fragile X syndrome exhibits defects in phagocytosis by innate immune cells
Reed M. O’Connor,Elizabeth F. Stone,Charlotte R. Wayne,Emily V. Marcinkevicius,Matt Ulgherait,Rebecca Delventhal,Meghan M Pantalia,Vanessa M. Hill,Clarice G. Zhou,Sophie F McAllister,Anna Chen,Jennifer S. Ziegenfuss,Wesley B. Grueber,Julie C. Canman,Mimi Shirasu-Hiza +14 more
TL;DR: It is found that Drosophila melanogaster Fmr1 mutants exhibit increased sensitivity to bacterial infection and decreased phagocytosis of bacteria by systemic immune cells, and a previously unrecognized role for FMR1 in regulating the activation of phagocytetic immune cells both in the body and the brain.
Journal ArticleDOI
Neuronal activity drives FMRP- and HSPG-dependent matrix metalloproteinase function required for rapid synaptogenesis
TL;DR: The data suggest that neuronal activity-induced, HSPG-dependent Mmp regulation drives activity-dependent synaptogenesis and that this is impaired in FXS.
Journal ArticleDOI
Transcriptional signatures of participant-derived neural progenitor cells and neurons implicate altered Wnt signaling in Phelan-McDermid syndrome and autism
Michael S. Breen,Andrew W. Browne,Gabriel E. Hoffman,Sofia Stathopoulos,Kristen J. Brennand,Joseph D. Buxbaum,Elodie Drapeau +6 more
TL;DR: Genome-wide RNA-sequencing in hiPSC-derived neural cells from individuals with PMS revealed both shared and distinct transcriptional signatures across hiPSS-NPCs and hiPSc-neurons, including many genes implicated in risk for ASD, as well as specific neurobiological pathways, including the Wnt pathway.
Journal ArticleDOI
Curvilinear Association Between Language Disfluency and FMR1 CGG Repeat Size Across the Normal, Intermediate, and Premutation Range.
Jessica Klusek,Anna Porter,Leonard J Abbeduto,Tatyana Adayev,Flora Tassone,Marsha R. Mailick,Anne Glicksman,Bridgette L. Tonnsen,Jane E. Roberts +8 more
TL;DR: Findings suggest CGG-dependent variation in language production ability, which was evident across individuals with and without CGG expansions on FMR1, and challenges prior theoretical work and suggests that a primary language deficit could account for elevated language disfluency in F MR1-associated conditions.
References
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