Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
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C9ORF72, implicated in amytrophic lateral sclerosis and frontotemporal dementia, regulates endosomal traffickingFrom animal models to human disease: a genetic approach for personalized medicine in ALSPathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disordersWild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.Abnormalities of Mitochondrial Dynamics in Neurodegenerative Diseases.Drosophila lines with mutant and wild type human TDP-43 replacing the endogenous gene reveals phosphorylation and ubiquitination in mutant lines in the absence of viability or lifespan defects.Linking RNA Dysfunction and Neurodegeneration in Amyotrophic Lateral Sclerosis.Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1.Investigating the role of filamin C in Belgian patients with frontotemporal dementia linked to GRN deficiency in FTLD-TDP brains.Mitochondrial dynamic abnormalities in amyotrophic lateral sclerosis.Rodent Models of Amyotrophic Lateral SclerosisMass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains.Two mutations G335D and Q343R within the amyloidogenic core region of TDP-43 influence its aggregation and inclusion formation.An insoluble frontotemporal lobar degeneration-associated TDP-43 C-terminal fragment causes neurodegeneration and hippocampus pathology in transgenic miceDisease animal models of TDP-43 proteinopathy and their pre-clinical applications.Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.FTD and ALS--translating mouse studies into clinical trials.Frontotemporal Lobar Degeneration: Mechanisms and Therapeutic Strategies.Advances in the Development of Disease-Modifying Treatments for Amyotrophic Lateral Sclerosis.TDP-43/FUS in motor neuron disease: Complexity and challenges.Disease-associated mutations of TDP-43 promote turnover of the protein through the proteasomal pathway.Rapamycin alleviates pathogenesis of a new Drosophila model of ALS-TDP.Mitochondrial quality control in amyotrophic lateral sclerosis: towards a common pathway?Adenoviral expression of TDP-43 and FUS genes and shRNAs for protein degradation pathways in rodent motoneurons in vitro and in vivo.Novel miR-b2122 regulates several ALS-related RNA-binding proteins.Heterogeneity of Matrin 3 in the developing and aging murine central nervous system.Towards a TDP-43-Based Biomarker for ALS and FTLD.TDP43 and RNA instability in amyotrophic lateral sclerosis.TDP-43 binds and transports G-quadruplex-containing mRNAs into neurites for local translation.From Mouse Models to Human Disease: An Approach for Amyotrophic Lateral Sclerosis
P2860
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P2860
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
description
2013 nî lūn-bûn
@nan
2013 թուականի Մարտին հրատարակուած գիտական յօդուած
@hyw
2013 թվականի մարտին հրատարակված գիտական հոդված
@hy
2013年の論文
@ja
2013年論文
@yue
2013年論文
@zh-hant
2013年論文
@zh-hk
2013年論文
@zh-mo
2013年論文
@zh-tw
2013年论文
@wuu
name
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@ast
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@en
type
label
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@ast
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@en
prefLabel
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@ast
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@en
P2093
P2860
P1476
Overexpression of ALS-associat ...... o wild-type human TDP-43 mice.
@en
P2093
Chantal Ceuterick-de Groote
Geert Joris
Jonathan Janssens
P2860
P2888
P356
10.1007/S12035-013-8427-5
P577
2013-03-10T00:00:00Z
P5875
P6179
1010950241