ArticleCell reports2023
Poly(GR) interacts with key stress granule factors promoting its assembly into cytoplasmic inclusions.
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Article in Cell reports, 2023. 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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Who cites it
20 citing papers in PubMed.
- Sigma-1 Receptor Mitigates Poly-GR-Associated Redox Dysregulation by Restoring NRF2/HO1 Signaling in C9orf72-Associated ALS.Neuromolecular medicine · 2026Article
- Hypothalamus Participates in Peripheral Sympathetic Hyperactivity After Acute Brain Injury by RegulatingMedComm · 2026Article
- Review
- Dysregulation of liquid-liquid phase separation: from molecular mechanisms to targeted therapies in cardiovascular diseases.Journal of nanobiotechnology · 2026Review
- Neuromodulatory role and therapeutic potential of N 6 -methyladenosine RNA methylation in neurodegenerative diseases.Neural regeneration research · 2026Article
- [Mechanisms of RNA-binding protein phase separation in steroid-associated necrosis of the femoral head].Zhong nan da xue xue bao. Yi xue ban = Journal of Central South University. Medical sciences · 2026Review
- Condensate protein aggregation in ALS/FTD is regulated by GGGGCC-repeat RNA scaffolds.Nature structural & molecular biology · 2026Article
- Mechanisms at the Intersection of lncRNA and m6A Biology.Non-coding RNA · 2026Review
- The mCells · 2025Review
- Machine learning-based proteomics profiling of ALS identifies downregulation of RPS29 that maintains protein homeostasis and STMN2 level.Communications biology · 2025Article
- Regulation of physiological and pathological condensates by molecular chaperones.The FEBS journal · 2025Review
- β-propeller protein-associated neurodegeneration protein WDR45 regulates stress granule disassembly via phase separation with Caprin-1.Nature communications · 2025Article
- Biomolecular condensates in immune cell fate.Nature reviews. Immunology · 2025Review
- Perillaldehyde Improves Parkinson-Like Deficits by Targeting G3BP Mediated Stress Granule Assembly in Preclinical Models.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025Article
- The epitranscriptome meets non-coding RNA: m6A-mediated regulation in oncogenesis and therapy.Frontiers in cell and developmental biology · 2025Review
- C9orf72-linked arginine-rich dipeptide repeats aggravate pathological phase separation of G3BP1.Proceedings of the National Academy of Sciences of the United States of America · 2024Article
- Co-Aggregation of TDP-43 with Other Pathogenic Proteins and Their Co-Pathologies in Neurodegenerative Diseases.International journal of molecular sciences · 2024Review
- The Effect of Dipeptide Repeat Proteins on FUS/TDP43-RNA Condensation in C9orf72 ALS/FTD.The journal of physical chemistry. B · 2024Article
- Liquid-liquid phase separation in diseases.MedComm · 2024Review
- ALS' Perfect Storm:Cells · 2024Review
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Authors and funding
25 authors.
Jinyoung ParkDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Yanwei WuDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Wei ShaoDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Tania F GendronDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA; Neurobiology of Disease Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Rochester, MN 55902, USA.
Sophie J F van der SpekDepartment of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA.
Grigorii SultanakhmetovDepartment of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA; Department of Biological Sciences, Graduate School of Science, Tokyo Metropolitan University, Tokyo, 1920397, Japan.
Avik BasuCenter for Network Systems Biology, Boston University School of Medicine, Boston, MA 02118, USA.
Paula Castellanos OteroDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Caroline J JonesDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Karen Jansen-WestDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Lillian M DaughrityDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Sadhna PhanseCenter for Network Systems Biology, Boston University School of Medicine, Boston, MA 02118, USA.
Giulia Del RossoDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Jimei TongDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Monica Castanedes-CaseyDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA.
Lulu JiangDepartment of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA.
Jenna LiberaDepartment of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA.
Björn OskarssonDepartment of Neurology, Mayo Clinic, Jacksonville, FL 32224, USA.
Dennis W DicksonDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA; Neurobiology of Disease Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Rochester, MN 55902, USA.
David W SandersDepartment of Chemical and Biological Engineering, Princeton University, Princeton, NJ 08544, USA.
Clifford P BrangwynneDepartment of Chemical and Biological Engineering, Princeton University, Princeton, NJ 08544, USA; Howard Hughes Medical Institute, Princeton, NJ 08544, USA.
Andrew EmiliCenter for Network Systems Biology, Boston University School of Medicine, Boston, MA 02118, USA.
Benjamin WolozinDepartment of Pharmacology and Experimental Therapeutics, Boston University School of Medicine, Boston, MA 02118, USA.
Leonard PetrucelliDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA; Neurobiology of Disease Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Rochester, MN 55902, USA. Electronic address: petrucelli.leonard@mayo.edu.
Yong-Jie ZhangDepartment of Neuroscience, Mayo Clinic, Jacksonville, FL 32224, USA; Neurobiology of Disease Graduate Program, Mayo Graduate School, Mayo Clinic College of Medicine, Rochester, MN 55902, USA. Electronic address: zhang.yongjie@mayo.edu.
Funding
TDP-43 Loss-of-Function: Biology to BiomarkersP01AG019724 · NIA · UNIVERSITY OF PENNSYLVANIA · PI Jennifer Merrilees · 2002 to 2026
$67.2MProject 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
$10.6MHuman Biomarkers CoreU54NS123743 · NINDS · STANFORD UNIVERSITY · PI GITLER, AARON D. · 2021 to 2025
$8.2MValidation 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
$6.5MNovel genetic modifiers of C9orf72 and Tau toxicityRF1AG062077 · NIA · MAYO CLINIC ARIZONA · PI FRYER, JOHN DAVID, PETRUCELLI, LEONARD · 2019 to 2019
$4.0MSystems-level functional proteomics analysis assemblies in Alzheimer's disease and mouse models of tauopathyRF1AG061706 · NIA · BOSTON UNIVERSITY MEDICAL CAMPUS · PI EMILI, ANDREW, WOLOZIN, BENJAMIN L · 2019 to 2019
$3.9MNuclear Pore Complex and as a Contributor to Tau Associated DementiaRF1AG062171 · NIA · JOHNS HOPKINS UNIVERSITY · PI PETRUCELLI, LEONARD, ROTHSTEIN, JEFFREY D · 2019 to 2019
$3.8MCircular RNAs and their interactions with RNA-binding proteins to modulate AD-related neuropathologyU01AG072577 · NIA · BOSTON UNIVERSITY MEDICAL CAMPUS · PI WOLOZIN, BENJAMIN L, ZHANG, XIAOLING · 2021 to 2025
$3.8MThe role of N6-methyladenosine modified RNA in Alzheimer's disease: Equipment SupplementR01AG080810 · NIA · BOSTON UNIVERSITY MEDICAL CAMPUS · PI Benjamin L Wolozin, Xiaoling Zhang · 2023 to 2026
$3.2MPoly(ADP-ribose) promotes the condensation and toxicity of C9orf72 arginine-rich dipeptide repeat proteinsR01NS117461 · NINDS · UNIVERSITY OF MIAMI SCHOOL OF MEDICINE · PI Tania France Gendron, Yongjie Zhang · 2021 to 2026
$2.9MGlycogen promotes protein aggregation in ALS and FTDR21NS127331 · NINDS · MAYO CLINIC JACKSONVILLE · PI ZHANG, YONGJIE · 2022 to 2022
$430kNIA NIH HHS P01 AG019724NIA NIH HHS R01 AG080810NIA NIH HHS RF1 AG061706NIA NIH HHS RF1 AG062077NIA NIH HHS RF1 AG062171NIA NIH HHS U01 AG072577NINDS NIH HHS P01 NS084974NINDS NIH HHS P01 NS099114NINDS NIH HHS R01 NS117461NINDS NIH HHS R21 NS127331NINDS NIH HHS R35 NS097273NINDS NIH HHS U54 NS123743
Abstract
C9orf72 repeat expansions are the most common genetic cause of frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Poly(GR) proteins are toxic to neurons by forming cytoplasmic inclusions that sequester RNA-binding proteins including stress granule (SG) proteins. However, little is known of the factors governing poly(GR) inclusion formation. Here, we show that poly(GR) infiltrates a finely tuned network of protein-RNA interactions underpinning SG formation. It interacts with G3BP1, the key driver of SG assembly and a protein we found is critical for poly(GR) inclusion formation. Moreover, we discovered that N
Indexed as
Amyotrophic Lateral SclerosisFrontotemporal DementiaAnimalsC9orf72 ProteinDNA HelicasesDNA Repeat ExpansionHeat-Shock ProteinsHumansInclusion BodiesMicePoly-ADP-Ribose Binding ProteinsRNARNA HelicasesRNA Recognition Motif ProteinsStress GranulesC9orf72 ProteinDNA HelicasesG3BP1 protein, humanHeat-Shock ProteinsPoly-ADP-Ribose Binding ProteinsRNARNA HelicasesRNA Recognition Motif Proteinsamyotrophic lateral sclerosischromosome 9 open reading frame 72CP: Molecular biologyCP: Neurosciencecytoplasmic poly(GR) inclusionsdipeptide repeat proteinsfrontotemporal dementiaG3BP1/2liquid-liquid phase separationm6A-modified RNAsYTHDF proteins
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