Evidence map›Paper›PMID 28005950›Full record

ArticlePloS one2016

CGG Repeats in the 5'UTR of FMR1 RNA Regulate Translation of Other RNAs Localized in the Same RNA Granules.

René Rovozzo, George Korza, Mei W Baker, Meng Li, Anita Bhattacharyya, Elisa Barbarese, John H Carson

Open access · goldAbstract read
In one paragraph

Article in PloS one, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 13 papers.

0numbers the graph read from it
0cells of the map it votes in
13citing papers in PubMed
2.4field-weighted citation impact, top 10% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

13 citing papers in PubMed, 26 citations in OpenAlex.

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4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

7 authors at 2 institutions in 1 country.

René RovozzoCenter for Cell Analysis and Modeling, UConn Health, Farmington, Connecticut, United States of America.
George KorzaDepartment of Molecular Biology and Biophysics, UConn Health, Farmington, Connecticut, United States of America.
Mei W BakerDepartments of Pediatrics, School of Medicine and Public Health, University of Wisconsin-Madison, Wisconsin State Laboratory of Hygiene, Newborn Screening Laboratory, Madison, Wisconsin, United States of America.
Meng LiWaisman Center, University of Wisconsin, Madison, Wisconsin, United States of America.
Anita BhattacharyyaWaisman Center, University of Wisconsin, Madison, Wisconsin, United States of America.
Elisa BarbareseDepartment of Neuroscience, UConn Health, Farmington, Connecticut, United States of America.
John H CarsonCenter for Cell Analysis and Modeling, UConn Health, Farmington, Connecticut, United States of America.
UConn Health · USUniversity of Wisconsin–Madison · US

Funding

Institutional Clinical and Translational Science AwardUL1TR000427 · NCATS · UNIVERSITY OF WISCONSIN-MADISON · PI DREZNER, MARC KENNETH · 2012 to 2016
$32.2M
VIRTUAL FCSP41RR013186 · NCRR · UNIVERSITY OF CONNECTICUT SCH OF MED/DNT · PI LOEW, LESLIE M · 1998 to 2011
$14.2M
OLIGODENDROCYTE ONTOGENY AND DIFFERENTIATIONR01NS019943 · NINDS · UNIVERSITY OF CONNECTICUT SCH OF MED/DNT · PI BARBARESE, ELISA M · 1985 to 2011
$3.1M
NCATS NIH HHS UL1 TR000427NCRR NIH HHS P41 RR013186NINDS NIH HHS R01 NS019943
6 · The paper itself

Abstract

CGG repeats in the 5'UTR of Fragile X Mental Retardation 1 (FMR1) RNA mediate RNA localization and translation in granules. Large expansions of CGG repeats (> 200 repeats) in FMR1, referred to as full mutations, are associated with fragile X syndrome (FXS). Smaller expansions (55-200 repeats), referred to as premutations, are associated with fragile X tremor ataxia syndrome (FXTAS) and fragile X premature ovarian insufficiency (FXPOI). TMPyP4 is a porphyrin ring compound that destabilizes CGG repeat RNA secondary structure. Here we show that exogenous CGG repeat RNA by itself, lacking the FMRP ORF, microinjected into hippocampal neurons is localized in RNA granules and inhibits translation of ARC RNA, which is localized in the same granules. TMPyP4 rescues translation of ARC RNA in granules. We also show that in human premutation fibroblasts with endogenous CGG repeat expansions in the FMR1 gene, translation of ARC RNA is inhibited and calcium homeostasis is disrupted and both phenotypes are rescued by TMPyP4. Inhibition of granule translation by expanded CGG repeats and rescue of granule translation by TMPy4, represent potential pathogenic mechanism and therapeutic strategy, respectively, for FXTAS and FXPOI.

Indexed as

Protein Biosynthesis5' Untranslated RegionsAnimalsCells, CulturedCytoskeletal ProteinsEmbryo, MammalianFemaleFibroblastsFragile X Messenger Ribonucleoprotein 1HippocampusHumansMiceMice, Inbred C57BLNerve Tissue ProteinsNeuronsPrimary Ovarian Insufficiency5' Untranslated Regionsactivity regulated cytoskeletal-associated proteinCytoskeletal ProteinsFMR1 protein, humanFragile X Messenger Ribonucleoprotein 1Nerve Tissue ProteinsRNA

Identifiers

PMID28005950
PMCPMC5179066
OpenAlexW2568876406

What OpenQuestion holds

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LicenceCC BY
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Registered trials

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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.