ArticleJournal of molecular biology2011
Initiation of translation of the FMR1 mRNA Occurs predominantly through 5'-end-dependent ribosomal scanning.
Article in Journal of molecular biology, 2011. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
20 citing papers in PubMed, 25 citations in OpenAlex.
- Size matters: Fighting repeat expansion size in fragile X syndrome using antisense oligonucleotides.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- A native function for RAN translation and CGG repeats in regulating fragile X protein synthesis.Nature neuroscience · 2020Article
- hnRNP Q Regulates Internal Ribosome Entry Site-MediatedMolecular and cellular biology · 2019Article
- Vagal Tone as a Putative Mechanism for Pragmatic Competence: An Investigation of Carriers of the FMR1 Premutation.Journal of autism and developmental disorders · 2019Article
- Curvilinear Association Between Language Disfluency andFrontiers in genetics · 2018Article
- RAN translation-What makes it run?Brain research · 2016Review
- CGG Repeat-Associated Non-AUG Translation Utilizes a Cap-Dependent Scanning Mechanism of Initiation to Produce Toxic Proteins.Molecular cell · 2016Article
- RNA-protein interactions in unstable microsatellite diseases.Brain research · 2014Review
- Repeat-associated non-AUG translation and its impact in neurodegenerative disease.Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics · 2014Review
- Expression of an expanded CGG-repeat RNA in a single pair of primary sensory neurons impairs olfactory adaptation in Caenorhabditis elegans.Human molecular genetics · 2014Article
- CNS expression of murine fragile X protein (FMRP) as a function of CGG-repeat size.Human molecular genetics · 2014Article
- Fragile X mental retardation protein expression in Alzheimer's disease.Frontiers in genetics · 2014Article
- Clinical and molecular implications of mosaicism in FMR1 full mutations.Frontiers in genetics · 2014Article
- CGG repeat-associated translation mediates neurodegeneration in fragile X tremor ataxia syndrome.Neuron · 2013Article
- Impaired activity-dependent FMRP translation and enhanced mGluR-dependent LTD in Fragile X premutation mice.Human molecular genetics · 2013Article
- Cap-dependent translation without base-by-base scanning of an messenger ribonucleic acid.Nucleic acids research · 2012Article
- Neurodegeneration the RNA way.Progress in neurobiology · 2012Review
- Fragile X syndrome: the FMR1 CGG repeat distribution among world populations.Annals of human genetics · 2012Review
- Decreased fragile X mental retardation protein expression underlies amygdala dysfunction in carriers of the fragile X premutation.Biological psychiatry · 2011Article
- Repeat associated non-ATG translation initiation: one DNA, two transcripts, seven reading frames, potentially nine toxic entities!PLoS genetics · 2011Review
Corrections and comments
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Authors and funding
3 authors at 1 institution in 1 country.
Funding
Abstract
The fragile X mental retardation 1 (FMR1) gene contains a CGG repeat within its 5' untranslated region (5'UTR) that, when expanded to 55-200 CGG repeats (premutation allele), can result in the late-onset neurodegenerative disorder, fragile X-associated tremor/ataxia syndrome. The CGG repeat is expected to form a highly stable secondary structure that is capable of inhibiting 5'-cap-dependent translation. Paradoxically, translation in vivo is only mildly impaired within the premutation range, suggesting that other modes of translation initiation may be operating. To address this issue, we translated in vitro a set of reporter mRNAs containing between 0 and 99 CGG repeats in either native (FMR1) or unrelated (heterologous) 5'UTR context. The 5'-cap dependence of translation was assessed by inserting a stable hairpin (HP) near the 5' end of the mRNAs. The results of the current studies indicate that translation initiation of the FMR1 mRNA occurs primarily by scanning, with little evidence of internal ribosome entry or shunting. Additionally, the efficiency of translation initiation depends on transcription start site selection, with the shorter 5'UTR (downstream transcription start site I) translating with greater efficiency compared to the longer mRNA (start site III) for all CGG-repeat elements studied. Lastly, an HP previously shown to block translation gave differing results depending on the 5'UTR context, in one case initiating translation from within the HP.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.