Literature DB >> 28103472

Microsatellite Expansion Diseases: Repeat Toxicity Found in Translation.

Fen-Biao Gao1, Joel D Richter2.   

Abstract

Sellier et al. (2017) show that translation of expanded CGG repeats in fragile X-associated tremor/ataxia syndrome is initiated at an upstream ACG near-cognate start codon. The resulting polyglycine-containing protein, but not repeat RNA, is pathogenic by disrupting the nuclear lamina.
Copyright © 2017 Elsevier Inc. All rights reserved.

Entities:  

Keywords:  ALS; C9ORF72; FMR1; FXTAS; RAN translation; frontotemporal dementia; microsatellite; neurodegeneration; nuclear lamina; repeats

Mesh：

Substances：

Year:  2017        PMID： 28103472     DOI： 10.1016/j.neuron.2017.01.001

Source DB:  PubMed          Journal:  Neuron        ISSN： 0896-6273            Impact factor:   18.688


  5 in total

1.  An explanation of the mechanisms underlying fragile X-associated premature ovarian insufficiency.

Authors:  Bruce I Rose; Samuel E Brown
Journal:  J Assist Reprod Genet       Date:  2020-05-06       Impact factor: 3.412

2.  Translation of GGC repeat expansions into a toxic polyglycine protein in NIID defines a novel class of human genetic disorders: The polyG diseases.

Authors:  Manon Boivin; Jianwen Deng; Véronique Pfister; Erwan Grandgirard; Mustapha Oulad-Abdelghani; Bastien Morlet; Frank Ruffenach; Luc Negroni; Pascale Koebel; Hugues Jacob; Fabrice Riet; Anke A Dijkstra; Kathryn McFadden; Wiley A Clayton; Daojun Hong; Hiroaki Miyahara; Yasushi Iwasaki; Jun Sone; Zhaoxia Wang; Nicolas Charlet-Berguerand
Journal:  Neuron       Date:  2021-04-21       Impact factor: 17.173

Review 3.  Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.

Authors:  Fen-Biao Gao; Sandra Almeida; Rodrigo Lopez-Gonzalez
Journal:  EMBO J       Date:  2017-09-15       Impact factor: 14.012

Review 4.  Clinical and mechanism advances of neuronal intranuclear inclusion disease.

Authors:  Yueqi Liu; Hao Li; Xuan Liu; Bin Wang; Hao Yang; Bo Wan; Miao Sun; Xingshun Xu
Journal:  Front Aging Neurosci       Date:  2022-09-13       Impact factor: 5.702

5.  Genome-wide characterization of microsatellite DNA in fishes: survey and analysis of their abundance and frequency in genome-specific regions.

Authors:  Yi Lei; Yu Zhou; Megan Price; Zhaobin Song
Journal:  BMC Genomics       Date:  2021-06-07       Impact factor: 3.969
  5 in total

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