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 cortical Cytoarchitecture in the Fmr1 knockout mouse
TL;DR: A comprehensive histological analysis of the cerebral cortex in Fmr1 KO mice showed the presence of astrogliosis characterized by an increase in GFAP expression and astrocyte hypertrophy, and insights towards possible mechanisms associated with FXS are provided.
Journal ArticleDOI
Integrated transcriptome analysis of human iPS cells derived from a fragile X syndrome patient during neuronal differentiation.
Ping Lu,Xiao-Long Chen,Yun Feng,Qiao Zeng,Cizhong Jiang,Xianmin Zhu,Guoping Fan,Guoping Fan,Zhigang Xue +8 more
TL;DR: It is demonstrated that the FMRP deficiency in FXS patients has significant impact on the gene expression patterns during development, which will help to discover potential targeting candidates for the cure of FXS symptoms.
Journal ArticleDOI
Shifting patterns of polyribosome accumulation at synapses over the course of hippocampal long-term potentiation.
Linnaea E. Ostroff,Deborah Watson,Guan Cao,Patrick H. Parker,Heather L Smith,Kristen M. Harris +5 more
TL;DR: Polyribosomes, the sites of local protein synthesis, are quantified in CA1 stratum radiatum dendrites and spines from postnatal day 15 rats to show that LTP induces a rapid, transient upregulation of large polyribosome in larger spines, and a persistent upregulation in the bases and necks of small spines.
Journal ArticleDOI
Cognitive and behavioral correlates of caudate subregion shape variation in fragile X syndrome
TL;DR: Investigation of topography within focal regions of the caudate nucleus associated with prefrontal circuitry and its link with aberrant behavior and intellect in fragile X syndrome supported the hypothesis that CN enlargement is related to abnormal orbitofrontal‐caudate and dorsolateral‐ caudate circuitry in FXS.
Journal ArticleDOI
Disruptive variants of CSDE1 associate with autism and interfere with neuronal development and synaptic transmission
Hui Guo,Hui Guo,Ying Li,Lu Shen,Tianyun Wang,Tianyun Wang,Xiangbin Jia,Lijuan Liu,Tao Xu,Mengzhu Ou,Kendra Hoekzema,Huidan Wu,Madelyn A. Gillentine,Cenying Liu,Hailun Ni,Pengwei Peng,Rongjuan Zhao,Yu Zhang,Chanika Phornphutkul,Alexander P.A. Stegmann,Carlos E. Prada,Robert J. Hopkin,Joseph T. Shieh,Kirsty McWalter,Kristin G. Monaghan,Peter M. van Hasselt,Koen L.I. van Gassen,Ting Bai,Min Long,Lin Han,Yingting Quan,Meilin Chen,Yaowen Zhang,Kuokuo Li,Qiumeng Zhang,Jieqiong Tan,Tengfei Zhu,Yaning Liu,Nan Pang,J Peng,Daryl A. Scott,Seema R. Lalani,Mahshid Azamian,Grazia M.S. Mancini,Darius J. Adams,Malin Kvarnung,Malin Kvarnung,Anna Lindstrand,Anna Lindstrand,Ann Nordgren,Ann Nordgren,Jonathan Pevsner,Jonathan Pevsner,Ikeoluwa A. Osei-Owusu,Ikeoluwa A. Osei-Owusu,Corrado Romano,Giuseppe Calabrese,Ornella Galesi,Jozef Gecz,E. Haan,Judith D. Ranells,Melissa Racobaldo,Magnus Nordenskjöld,Magnus Nordenskjöld,Suneeta Madan-Khetarpal,Jessica Sebastian,Susie Ball,Xiaobing Zou,Jingping Zhao,Zhengmao Hu,Fan Xia,Fan Xia,Pengfei Liu,Pengfei Liu,Jill A. Rosenfeld,Bert B.A. de Vries,Raphael Bernier,Zhi-Qing David Xu,Honghui Li,Wei Xie,Robert B. Hufnagel,Evan E. Eichler,Kun Xia +82 more
TL;DR: A new autism-related syndrome is defined and the functional role of CSDE1 in synapse development and synaptic transmission is highlighted, which is linked to overgrowth of neurites and abnormal dendritic spine morphology/synapse formation and impaired synaptic transmission.
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