ArticleNature neuroscience2019
Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.
Erratum issuedAbstract read
In one paragraph
Article in Nature neuroscience, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 60 papers.
0numbers the graph read from it
0cells of the map it votes in
60citing papers in PubMed
7.8field-weighted citation impact, top 2% of its field
What it found
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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.
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.
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Who cites it
60 citing papers in PubMed, 81 citations in OpenAlex.
- PAICS mediates DNA damage and cerebellar neuronal loss in C9orf72 amyotrophic lateral sclerosis.Brain : a journal of neurology · 2026Article
- Insights into X-linked Dystonia-Parkinsonism and Spinocerebellar Ataxia type 36 Through the Lens of Amyotrophic Lateral Sclerosis/Frontotemporal Dementia.Molecular neurobiology · 2026Review
- Article
- Increased neuronal activity restores circadian function in Drosophila models of C9orf72-ALS/FTD.iScience · 2026Article
- Disrupted sleep-wake cycles and circadian rhythms in aFrontiers in neuroscience · 2026Article
- Exploring the versatility ofFly · 2025Review
- Article
- Drosophila Topoisomerase 3β binds to mRNAs in vivo, contributes to their localization and stability, and counteracts premature aging.PloS one · 2025Article
- Transgenic sensors reveal compartment-specific effects of aggregation-prone proteins on subcellular proteostasis during aging.Cell reports methods · 2024Article
- Tau is required for glial lipid droplet formation and resistance to neuronal oxidative stress.Nature neuroscience · 2024Article
- Ribosomal quality control factors inhibit repeat-associated non-AUG translation from GC-rich repeats.Nucleic acids research · 2024Article
- TDP-43 impairs sleep inScience advances · 2024Article
- A comparison study of pathological features and drug efficacy between Drosophila models of C9orf72 ALS/FTD.Molecules and cells · 2024Article
- Role of C9orf72 hexanucleotide repeat expansions in ALS/FTD pathogenesis.Frontiers in molecular neuroscience · 2024Review
- Pathological insights from amyotrophic lateral sclerosis animal models: comparisons, limitations, and challenges.Translational neurodegeneration · 2023Review
- A monocarboxylate transporter rescues frontotemporal dementia and Alzheimer's disease models.PLoS genetics · 2023Article
- Dissecting the roles of EIF4G homologs reveals DAP5 as a modifier of CGG repeat-associated toxicity in a Drosophila model of FXTAS.Neurobiology of disease · 2023Article
- FUS regulates RAN translation through modulating the G-quadruplex structure of GGGGCC repeat RNA ineLife · 2023Article
- Autolysosomal exocytosis of lipids protect neurons from ferroptosis.The Journal of cell biology · 2023Article
- Review
Corrections and comments
- Erratum issued
Authors and funding
14 authors at 3 institutions in 1 country.
Lindsey D GoodmanNeuroscience Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
Mercedes PrudencioDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.ORCID http://orcid.org/0000-0002-4894-4858
Nicholas J KramerNeuroscience Graduate Program, Stanford University School of Medicine, Stanford, CA, USA.ORCID http://orcid.org/0000-0003-4557-8343
Luis F Martinez-RamirezDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.ORCID http://orcid.org/0000-0001-9619-1689
Ananth R SrinivasanDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.
Matthews LanDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.
Michael J ParisiDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.
Yongqing ZhuDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.
Jeannie ChewDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
Casey N CookDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
Amit BersonDepartment of Biology, University of Pennsylvania, Philadelphia, PA, USA.
Aaron D GitlerDepartment of Genetics, Stanford University School of Medicine, Stanford, CA, USA.ORCID http://orcid.org/0000-0001-8603-1526
Leonard PetrucelliDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL, USA.
Nancy M BoniniNeuroscience Graduate Group, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA. nbonini@sas.upenn.edu.ORCID http://orcid.org/0000-0003-0226-5291
University of Pennsylvania · USMayo Clinic in Florida · USStanford University · US
Funding
Project 3: Neuropathology of the multi-proteinopathy of c9FTD/ALS.P01NS084974 · NINDS · MAYO CLINIC JACKSONVILLE · PI PETRUCELLI, LEONARD · 2014 to 2024
$17.6MExpanding insights into FTD disease mechanismsR35NS097273 · NINDS · MAYO CLINIC JACKSONVILLE · PI PETRUCELLI, LEONARD · 2017 to 2024
$10.6MDrosophila Transgenic RNAi Resource ProjectR01GM084947 · NIGMS · HARVARD MEDICAL SCHOOL · PI PERRIMON, NORBERT · 2008 to 2019
$9.8MInnovating Yeast and Human Genetics Approaches to Define Mechanisms of Neurodegenerative DiseaseR35NS097263 · NINDS · STANFORD UNIVERSITY · PI GITLER, AARON D. · 2017 to 2024
$8.6MSYSTEMS AND INTEGRATIVE BIOLOGYT32GM007517 · NIGMS · UNIVERSITY OF PENNSYLVANIA · PI GOLD, JOSHUA I · 1985 to 2017
$8.0MValidation of r(G4C2)exp-binding small molecules in vivo and c9FTD/ALS biomarker developmentP01NS099114 · NINDS · UNIVERSITY OF FLORIDA · PI ROTHSTEIN, JEFFREY D · 2017 to 2021
$6.5MMolecular Genetic Insight into Neurodegenerative Disease from DrosophilaR35NS097275 · NINDS · UNIVERSITY OF PENNSYLVANIA · PI BONINI, NANCY M · 2017 to 2024
$6.0MEpigenetic Changes associated with Neurodegenerative DiseasesR01NS078283 · NINDS · UNIVERSITY OF PENNSYLVANIA · PI BERGER, SHELLEY L, BONINI, NANCY M · 2012 to 2016
$3.3MSYNAPTOBREVIN AND NEUROTRANSMITTER RELEASEF32NS009727 · NINDS · STANFORD UNIVERSITY · PI DEITCHER, DAVID L · 1994 to 1996
–NIGMS NIH HHS R01 GM084947NIGMS NIH HHS T32 GM007517NINDS NIH HHS F32 NS009727NINDS NIH HHS P01 NS084974NINDS NIH HHS P01 NS099114NINDS NIH HHS R01 NS078283NINDS NIH HHS R35 NS097263NINDS NIH HHS R35 NS097273NINDS NIH HHS R35 NS097275
Abstract
An expanded GGGGCC hexanucleotide of more than 30 repeats (termed (G4C2)
Indexed as
AnimalsC9orf72 ProteinDNA Repeat ExpansionDrosophila melanogasterFrontotemporal DementiaGene Expression RegulationHumansMiceNuclear ProteinsTranscription FactorsC9orf72 ProteinLEO1 protein, humanNuclear ProteinsPAF1 protein, humanTranscription Factors
Identifiers
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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.