Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. - Wikidata - wikimedia - TriplyDB

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.

Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.

http://www.wikidata.org/entity/Q30532680

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From animal models to human disease: a genetic approach for personalized medicine in ALS Mechanisms of FUS mutations in familial amyotrophic lateral sclerosis Dysregulated axonal RNA translation in amyotrophic lateral sclerosis Toward precision medicine in amyotrophic lateral sclerosis FUS-mediated regulation of alternative RNA processing in neurons: insights from global transcriptome analysis The potential of pathological protein fragmentation in blood-based biomarker development for dementia - with emphasis on Alzheimer's disease Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis HDAC4-myogenin axis as an important marker of HD-related skeletal muscle atrophy Early lethality and neuronal proteinopathy in mice expressing cytoplasm-targeted FUS that lacks the RNA recognition motif.Pathogenesis of FUS-associated ALS and FTD: insights from rodent models Systems biology of neurodegenerative diseases FUS Interacts with HSP60 to Promote Mitochondrial Damage Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.Characterization of genetic loss-of-function of Fus in zebrafish.Motor neuron intrinsic and extrinsic mechanisms contribute to the pathogenesis of FUS-associated amyotrophic lateral sclerosis.ALS-linked FUS exerts a gain of toxic function involving aberrant p38 MAPK activation.An evaluation of a SVA retrotransposon in the FUS promoter as a transcriptional regulator and its association to ALS Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis.Functional interaction between FUS and SMN underlies SMA-like splicing changes in wild-type hFUS mice.A comprehensive analysis of rare genetic variation in amyotrophic lateral sclerosis in the UK Activity-dependent FUS dysregulation disrupts synaptic homeostasis The Impact of Endurance Training on Human Skeletal Muscle Memory, Global Isoform Expression and Novel Transcripts Characterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq.ALS-associated FUS mutations result in compromised FUS alternative splicing and autoregulation.Model systems of motor neuron diseases as a platform for studying pathogenic mechanisms and searching for therapeutic agents.Severe respiratory changes at end stage in a FUS-induced disease state in adult rats.Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS.ALS-associated mutant FUS induces selective motor neuron degeneration through toxic gain of function.The wobbler mouse, an ALS animal model.Toxic gain of function from mutant FUS protein is crucial to trigger cell autonomous motor neuron loss.Fused in sarcoma (FUS) protein lacking nuclear localization signal (NLS) and major RNA binding motifs triggers proteinopathy and severe motor phenotype in transgenic mice.FUS-linked essential tremor associated with motor dysfunction in Drosophila.FUS/TLS acts as an aggregation-dependent modifier of polyglutamine disease model mice.Changes in the Expression of FUS/TLS in Spinal Cords of SOD1 G93A Transgenic Mice and Correlation with Motor-Neuron Degeneration.The RNA-binding protein Fus directs translation of localized mRNAs in APC-RNP granules.Motor neuron disease, TDP-43 pathology, and memory deficits in mice expressing ALS-FTD-linked UBQLN2 mutations.FUS Mislocalization and Vulnerability to DNA Damage in ALS Patients Derived hiPSCs and Aging Motoneurons.ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects.TARDBP and FUS mutations associated with amyotrophic lateral sclerosis: summary and update.Protein aggregation in amyotrophic lateral sclerosis.

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Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion.

http://www.wikidata.org/entity/Q30532680

description

2012 nî lūn-bûn

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2012 թուականի Սեպտեմբերին հրատարակուած գիտական յօդուած

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2012 թվականի սեպտեմբերին հրատարակված գիտական հոդված

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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Overexpression of human wild-t ...... e- and dose-dependent fashion.

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P2860

Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import

A new subtype of frontotemporal lobar degeneration with FUS pathology

FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration

Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS

Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis

Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6

FUS pathology in basophilic inclusion body disease

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