Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans - Wikidata - awgldk - TriplyDB

Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans

Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans

http://www.wikidata.org/entity/Q35070967

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From animal models to human disease: a genetic approach for personalized medicine in ALS There has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALS Oxidative Stress in Caenorhabditis elegans: Protective Effects of Spartin C9orf72's Interaction with Rab GTPases-Modulation of Membrane Traffic and Autophagy Mechanisms of aging-related proteinopathies in Caenorhabditis elegans The ALS/FTLD associated protein C9orf72 associates with SMCR8 and WDR41 to regulate the autophagy-lysosome pathway.Loss of C9orf72 Enhances Autophagic Activity via Deregulated mTOR and TFEB Signaling Loss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune disease Bioinformatics Data Mining Approach Suggests Coexpression of AGTPBP1 with an ALS-linked Gene C9orf72.C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Molecular network analysis suggests a logical hypothesis for the pathological role of c9orf72 in amyotrophic lateral sclerosis/frontotemporal dementia.C9orf72 amyotrophic lateral sclerosis and frontotemporal dementia: gain or loss of function?RNA-protein interactions in unstable microsatellite diseases.Aberrant RNA homeostasis in amyotrophic lateral sclerosis: potential for new therapeutic targets?ALS mutant FUS proteins are recruited into stress granules in induced pluripotent stem cell-derived motoneurons Distribution of dipeptide repeat proteins in cellular models and C9orf72 mutation cases suggests link to transcriptional silencing C9ORF72 expression and cellular localization over mouse development.C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.Conserved pharmacological rescue of hereditary spastic paraplegia-related phenotypes across model organisms.C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice.Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs Forward Genetic Screen in Caenorhabditis elegans Suggests F57A10.2 and acp-4 As Suppressors of C9ORF72 Related Phenotypes.FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons.C9ORF72 hexanucleotide repeats in behavioral and motor neuron disease: clinical heterogeneity and pathological diversity.Worming forward: amyotrophic lateral sclerosis toxicity mechanisms and genetic interactions in Caenorhabditis elegans.Use of Caenorhabditis elegans as a model to study Alzheimer's disease and other neurodegenerative diseases.Development of Therapeutics for C9ORF72 ALS/FTD-Related Disorders.C9ORF72 Regulates Stress Granule Formation and Its Deficiency Impairs Stress Granule Assembly, Hypersensitizing Cells to Stress.Genetics of FTLD: overview and what else we can expect from genetic studies.Insights into the pathogenic mechanisms of Chromosome 9 open reading frame 72 (C9orf72) repeat expansions.Simple animal models for amyotrophic lateral sclerosis drug discovery.ALS and FTD: an epigenetic perspective.Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity.R Loops and Links to Human Disease.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Oligonucleotide therapies: the future of amyotrophic lateral sclerosis treatment?Microglia and C9orf72 in neuroinflammation and ALS and frontotemporal dementia.C9orf72 is differentially expressed in the central nervous system and myeloid cells and consistently reduced in C9orf72, MAPT and GRN mutation carriers.

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Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans

http://www.wikidata.org/entity/Q35070967

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2013 nî lūn-bûn

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2013 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

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2013 թվականի դեկտեմբերին հրատարակված գիտական հոդված

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2013年の論文

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2013年論文

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2013年論文

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2013年論文

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2013年論文

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Deletion of C9ORF72 results in ...... ress sensitivity in C. elegans

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J Alex Parker

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Martine Therrien

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Discovery of Novel DENN Proteins: Implications for the Evolution of Eukaryotic Intracellular Membrane Structures and Human Disease

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

The product of C9orf72, a gene strongly implicated in neurodegeneration, is structurally related to DENN Rab-GEFs

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

Multiple sequence alignment with the Clustal series of programs

Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans

Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio

TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans

FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis

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