TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97. - Test2 - elhamkhani - TriplyDB

TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

http://www.wikidata.org/entity/Q33932237

about

Altered ribostasis: RNA-protein granules in degenerative disorders Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS Exome sequencing reveals VCP mutations as a cause of familial ALS Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS Model systems of protein-misfolding diseases reveal chaperone modifiers of proteotoxicity Prion-like domains as epigenetic regulators, scaffolds for subcellular organization, and drivers of neurodegenerative disease Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis Model organisms in the fight against muscular dystrophy: lessons from drosophila and Zebrafish From neurodevelopment to neurodegeneration: the interaction of neurofibromin and valosin-containing protein/p97 in regulation of dendritic spine formation Does a loss of TDP-43 function cause neurodegeneration?Cardiac-Restricted Expression of VCP/TER94 RNAi or Disease Alleles Perturbs Drosophila Heart Structure and Impairs Function.Regulation of protein quality control by UBE4B and LSD1 through p53-mediated transcription Hyperphosphorylation as a defense mechanism to reduce TDP-43 aggregation FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis Frontotemporal dementia: a bridge between dementia and neuromuscular disease Drosophila as an In Vivo Model for Human Neurodegenerative Disease Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila Amino acid analog toxicity in primary rat neuronal and astrocyte cultures: implications for protein misfolding and TDP-43 regulation The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.Native functions of the androgen receptor are essential to pathogenesis in a Drosophila model of spinobulbar muscular atrophy.Expansive gene transfer in the rat CNS rapidly produces amyotrophic lateral sclerosis relevant sequelae when TDP-43 is overexpressed.VCP is essential for mitochondrial quality control by PINK1/Parkin and this function is impaired by VCP mutations.Protein Homeostasis in Amyotrophic Lateral Sclerosis: Therapeutic Opportunities?Drosophila Valosin-Containing Protein is required for dendrite pruning through a regulatory role in mRNA metabolism A Mighty "Protein Extractor" of the Cell: Structure and Function of the p97/CDC48 ATPase.Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model The Role of the Heat Shock Protein B8 (HSPB8) in Motoneuron Diseases.TDP-43 regulates Drosophila neuromuscular junctions growth by modulating Futsch/MAP1B levels and synaptic microtubules organization Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS Getting folded: chaperone proteins in muscle development, maintenance and disease.Nuclear TAR DNA-binding protein 43: A new target for amyotrophic lateral sclerosis treatment Derlin-1 regulates mutant VCP-linked pathogenesis and endoplasmic reticulum stress-induced apoptosis.The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease.The complexities of p97 function in health and disease A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant Hsp104 suppresses polyglutamine-induced degeneration post onset in a drosophila MJD/SCA3 model.A fruitful endeavor: modeling ALS in the fruit fly

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P2860

TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.

http://www.wikidata.org/entity/Q33932237

description

2010 nî lūn-bûn

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2010 թուականի Յունիսին հրատարակուած գիտական յօդուած

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2010 թվականի հունիսին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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JNEUROSCI.5894-09.2010

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TDP-43 mediates degeneration i ...... aused by mutations in VCP/p97.

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Gillian P Ritson

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Jake B Guinto

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Virginia M-Y Lee

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P2860

Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs

Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis

Targeted gene expression as a means of altering cell fates and generating dominant phenotypes

VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD

Splicing factors SRp20 and 9G8 promote the nucleocytoplasmic export of mRNA

TDP-43, the signature protein of FTLD-U, is a neuronal activity-responsive factor

TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis

Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy

Diverse functions with a common regulator: ubiquitin takes command of an AAA ATPase

HDAC6 rescues neurodegeneration and provides an essential link between autophagy and the UPS

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