ALS/FTD-Associated C9ORF72 Repeat RNA Promotes Phase Transitions In Vitro and in Cells

scientific article published in December 2017

ALS/FTD-Associated C9ORF72 Repeat RNA Promotes Phase Transitions In Vitro and in Cells is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1016/J.CELREP.2017.11.093
P932PMC publication ID5741101
P698PubMed publication ID29262335

P50authorPavel IvanovQ38322661
P2093author name stringPaul J Anderson
Marta M Fay
P2860cites workHighly prevalent putative quadruplex sequence motifs in human DNAQ24529050
Prevalence of quadruplexes in the human genomeQ24529051
Stress granule assembly is mediated by prion-like aggregation of TIA-1Q24559953
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense interventionQ24562489
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALSQ24629495
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALSQ24633692
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTDQ24634583
Stress granules and processing bodies are dynamically linked sites of mRNP remodelingQ24678779
There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSQ26750577
G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunitsQ27309029
ATPase-Modulated Stress Granules Contain a Diverse Proteome and Substructure.Q27936710
Phase separation in biology; functional organization of a higher orderQ28071484
Phase separation by low complexity domains promotes stress granule assembly and drives pathological fibrillizationQ28588090
Cell-free formation of RNA granules: low complexity sequence domains form dynamic fibers within hydrogelsQ29614781
Dynamic shuttling of TIA-1 accompanies the recruitment of mRNA to mammalian stress granulesQ30014821
RNA phase transitions in repeat expansion disordersQ30224330
C9orf72 nucleotide repeat structures initiate molecular cascades of disease.Q33715387
Angiogenin-induced tRNA-derived stress-induced RNAs promote stress-induced stress granule assemblyQ33796328
Free mRNA in excess upon polysome dissociation is a scaffold for protein multimerization to form stress granulesQ33983718
Vgl1, a multi-KH domain protein, is a novel component of the fission yeast stress granules required for cell survival under thermal stressQ34246800
Mutant FUS proteins that cause amyotrophic lateral sclerosis incorporate into stress granulesQ34308339
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.Q34326849
Discovery of a biomarker and lead small molecules to target r(GGGGCC)-associated defects in c9FTD/ALS.Q34512451
Repeat associated non-ATG (RAN) translation: new starts in microsatellite expansion disordersQ34543709
G-quadruplex structures contribute to the neuroprotective effects of angiogenin-induced tRNA fragmentsQ34793223
Triplet repeat RNA structure and its role as pathogenic agent and therapeutic targetQ35633648
Macromolecular crowding regulates assembly of mRNA stress granules after osmotic stress: new role for compatible osmolytesQ35709848
The disordered P granule protein LAF-1 drives phase separation into droplets with tunable viscosity and dynamicsQ35740324
Formation and Maturation of Phase-Separated Liquid Droplets by RNA-Binding ProteinsQ36172705
Residue-by-Residue View of In Vitro FUS Granules that Bind the C-Terminal Domain of RNA Polymerase IIQ36172715
ALS/FTD Mutation-Induced Phase Transition of FUS Liquid Droplets and Reversible Hydrogels into Irreversible Hydrogels Impairs RNP Granule FunctionQ36320469
The disease-associated r(GGGGCC)n repeat from the C9orf72 gene forms tract length-dependent uni- and multimolecular RNA G-quadruplex structuresQ36742244
Repeat-associated non-ATG (RAN) translation in neurological diseaseQ37195032
Distinct stages in stress granule assembly and disassemblyQ37238978
RNA Controls PolyQ Protein Phase Transitions.Q37573060
Repeat expansion disease: progress and puzzles in disease pathogenesisQ37696972
RNA G-quadruplexes are globally unfolded in eukaryotic cells and depleted in bacteriaQ37721983
Stress granules and cell signaling: more than just a passing phase?Q38136844
Liquid-liquid phase separation in biologyQ38257388
Stress-specific differences in assembly and composition of stress granules and related fociQ38720884
Relationship between C9orf72 repeat size and clinical phenotypeQ39188186
Cell-free formation of RNA granules: bound RNAs identify features and components of cellular assemblies.Q39349822
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule DynamicsQ40287573
Retinoblastoma susceptibility genes contain 5' sequences with a high propensity to form guanine-tetrad structuresQ40411340
ALS Mutations Disrupt Phase Separation Mediated by α-Helical Structure in the TDP-43 Low-Complexity C-Terminal Domain.Q41036092
Interaction of human telomeric DNA with N-methyl mesoporphyrin IX.Q41986001
C9orf72 FTLD/ALS-associated Gly-Ala dipeptide repeat proteins cause neuronal toxicity and Unc119 sequestrationQ42003533
C9orf72 hexanucleotide repeat associated with amyotrophic lateral sclerosis and frontotemporal dementia forms RNA G-quadruplexesQ42424866
De Novo Missense Mutations in DHX30 Impair Global Translation and Cause a Neurodevelopmental Disorder.Q46493120
rG4-seq reveals widespread formation of G-quadruplex structures in the human transcriptomeQ48091155
A Liquid-to-Solid Phase Transition of the ALS Protein FUS Accelerated by Disease Mutation.Q53368534
Selective interactions of cationic porphyrins with G-quadruplex structuresQ74502236
P4510describes a project that usesImageJQ1659584
P433issue12
P921main subjectamyotrophic lateral sclerosisQ206901
P304page(s)3573-3584
P577publication date2017-12-01
P1433published inCell ReportsQ5058165
P1476titleALS/FTD-Associated C9ORF72 Repeat RNA Promotes Phase Transitions In Vitro and in Cells
P478volume21

Reverse relations

cites work (P2860)
Q64248184Aberrant deposition of stress granule-resident proteins linked to C9orf72-associated TDP-43 proteinopathy
Q97069749C9orf72 arginine-rich dipeptide repeats inhibit UPF1-mediated RNA decay via translational repression
Q64948055C9orf72-mediated ALS and FTD: multiple pathways to disease.
Q57468042Differences between acute and chronic stress granules, and how these differences may impact function in human disease
Q60017423Emerging Roles for Intermolecular RNA-RNA Interactions in RNP Assemblies
Q92534777Mechanisms of Immune Activation by c9orf72-Expansions in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia
Q92755838Perspectives for Applying G-Quadruplex Structures in Neurobiology and Neuropharmacology
Q89628567RNA Droplets
Q60017421RNA self-assembly contributes to stress granule formation and defining the stress granule transcriptome
Q49905433Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome.
Q102131196SARS-CoV-2 nucleocapsid protein phase-separates with RNA and with human hnRNPs
Q90468301Stress granules and neurodegeneration
Q90021829The Hairpin Form of r(G4C2)exp in c9ALS/FTD Is Repeat-Associated Non-ATG Translated and a Target for Bioactive Small Molecules
Q88650023The Role of RNA in Biological Phase Separations
Q90322407The involvement of stress granules in aging and aging-associated diseases
Q92095194The regulation and functions of DNA and RNA G-quadruplexes
Q59798011UPA-seq: prediction of functional lncRNAs using differential sensitivity to UV crosslinking
Q56535326Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism

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