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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Altered neuronal and circuit excitability in Fragile X Syndrome
TL;DR: The current state of knowledge of the molecular, synaptic, and circuit-level mechanisms underlying hyperexcitability and their contributions to the FXS phenotypes are reviewed.
The Pathophysiology of Fragile X (and What It Teaches Us about Synapses)
TL;DR: Deeper insight is yielded into how synaptic protein synthesis and plasticity are regulated by experience and ways that altered signaling and synaptic function can be corrected in fragile X are suggested.
Journal ArticleDOI
RNA-binding proteins in human genetic disease.
TL;DR: The authors review the role of RBPs in human genetic disorders, both Mendelian and somatic, discuss the molecular mechanisms of disease and highlight emerging therapeutic interventions that target RBPs.
Journal ArticleDOI
Muscleblind-like 2-Mediated Alternative Splicing in the Developing Brain and Dysregulation in Myotonic Dystrophy
Konstantinos Charizanis,Kuang-Yung Lee,Kuang-Yung Lee,Ranjan Batra,Marianne Goodwin,Chaolin Zhang,Yuan Yuan,Lily Shiue,Melissa S. Cline,Marina M. Scotti,Guangbin Xia,Ashok V. Kumar,Tetsuo Ashizawa,H. Brent Clark,Takashi Kimura,Masanori P. Takahashi,Harutoshi Fujimura,Kenji Jinnai,Hiroo Yoshikawa,Mário Gomes-Pereira,Geneviève Gourdon,Noriaki Sakai,Seiji Nishino,Thomas C. Foster,Manuel Ares,Robert B. Darnell,Maurice S. Swanson +26 more
TL;DR: It is reported that Mbnl2 knockout mice develop several DM-associated central nervous system (CNS) features including abnormal REM sleep propensity and deficits in spatial memory.
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Hepatitis C Virus RNA Functionally Sequesters miR-122
Joseph M. Luna,Joseph M. Luna,Troels K. H. Scheel,Troels K. H. Scheel,Tal Danino,Tal Danino,Katharina S. Shaw,Aldo Mele,John J. Fak,Eiko Nishiuchi,Constantin N. Takacs,Maria Teresa Catanese,Ype P. de Jong,Ype P. de Jong,Ira M. Jacobson,Charles M. Rice,Robert B. Darnell +16 more
TL;DR: It is proposed that HCV-induced miR-122 inhibition by HCV RNA may result in global de-repression of host mi R-122 targets, providing an environment fertile for the long-term oncogenic potential of HCV.
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