C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
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From animal models to human disease: a genetic approach for personalized medicine in ALSThere has been an awakening: Emerging mechanisms of C9orf72 mutations in FTD/ALSGolgi Fragmentation in ALS Motor Neurons. New Mechanisms Targeting Microtubules, Tethers, and Transport VesiclesALS Patient Stem Cells for Unveiling Disease Signatures of Motoneuron Susceptibility: Perspectives on the Deadly Mitochondria, ER Stress and Calcium TriadC9orf72's Interaction with Rab GTPases-Modulation of Membrane Traffic and AutophagyLoss of C9ORF72 impairs autophagy and synergizes with polyQ Ataxin-2 to induce motor neuron dysfunction and cell deathA C9ORF72/SMCR8-containing complex regulates ULK1 and plays a dual role in autophagyThe 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 SignalingLoss-of-function mutations in the C9ORF72 mouse ortholog cause fatal autoimmune diseaseC9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromesMouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaCell-type specific differences in promoter activity of the ALS-linked C9orf72 mouse ortholog.C9ORF72 expression and cellular localization over mouse development.Golgi fragmentation in amyotrophic lateral sclerosis, an overview of possible triggers and consequences.Novel clinical associations with specific C9ORF72 transcripts in patients with repeat expansions in C9ORF72.C9orf72 ablation causes immune dysregulation characterized by leukocyte expansion, autoantibody production, and glomerulonephropathy in mice.Maturation and electrophysiological properties of human pluripotent stem cell-derived oligodendrocytesGain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAsComparative interactomics analysis of different ALS-associated proteins identifies converging molecular pathwaysStage-specific functions of Semaphorin7A during adult hippocampal neurogenesis rely on distinct receptorsEpidemiology and molecular mechanism of frontotemporal lobar degeneration/amyotrophic lateral sclerosis with repeat expansion mutation in C9orf72.Old versus New Mechanisms in the Pathogenesis of ALS.New Insights on the Mechanisms of Disease Course Variability in ALS from Mutant SOD1 Mouse Models.Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Dynamic expression of the mouse orthologue of the human amyotropic lateral sclerosis associated gene C9orf72 during central nervous system development and neuronal differentiation.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.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.Nuclear transport dysfunction: a common theme in amyotrophic lateral sclerosis and frontotemporal dementia.ALS and FTD: an epigenetic perspective.Pathogenic determinants and mechanisms of ALS/FTD linked to hexanucleotide repeat expansions in the C9orf72 gene.Decoding ALS: from genes to mechanism.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.C9orf72: At the intersection of lysosome cell biology and neurodegenerative disease.New developments in RAN translation: insights from multiple diseases.Motoneuron Disease: Basic Science.TDP-43 in the spectrum of MND-FTLD pathologies.
P2860
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P2860
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
description
2015 nî lūn-bûn
@nan
2015年の論文
@ja
2015年論文
@yue
2015年論文
@zh-hant
2015年論文
@zh-hk
2015年論文
@zh-mo
2015年論文
@zh-tw
2015年论文
@wuu
2015年论文
@zh
2015年论文
@zh-cn
name
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@ast
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@en
type
label
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@ast
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@en
prefLabel
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@ast
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@en
P2093
P2860
P50
P356
P1433
P1476
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
@en
P2093
Anna M Blokhuis
Caroline A C Zundel
Derek J Blake
Henk-Jan Westeneng
Jan H Veldink
Leonard H van den Berg
Margo L Terpstra
Raymond D Schellevis
Renata Vieira de Sá
P2860
P304
P356
10.1002/ANA.24453
P577
2015-06-05T00:00:00Z