Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.
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Genetic Correction of SOD1 Mutant iPSCs Reveals ERK and JNK Activated AP1 as a Driver of Neurodegeneration in Amyotrophic Lateral Sclerosis.Mutant TDP-43 does not impair mitochondrial bioenergetics in vitro and in vivoPathogenic p62/SQSTM1 mutations impair energy metabolism through limitation of mitochondrial substrates.Mitochondrial hyperpolarization in iPSC-derived neurons from patients of FTDP-17 with 10+16 MAPT mutation leads to oxidative stress and neurodegeneration.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.Modeling the C9ORF72 repeat expansion mutation using human induced pluripotent stem cells.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral SclerosisPhase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.The Enigmatic Role of C9ORF72 in AutophagyFailure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.Axonal Degeneration during Aging and Its Functional Role in Neurodegenerative DisordersAutophagy Dysregulation in ALS: When Protein Aggregates Get Out of Hand.Roles of sigma-1 receptors on mitochondrial functions relevant to neurodegenerative diseases.Rethinking Unconventional Translation in Neurodegeneration.Sense-encoded poly-GR dipeptide repeat proteins correlate to neurodegeneration and uniquely co-localize with TDP-43 in dendrites of repeat-expanded C9orf72 amyotrophic lateral sclerosis.Converging Mechanisms of p53 Activation Drive Motor Neuron Degeneration in Spinal Muscular Atrophy.RNA-binding proteins in neurodegeneration: mechanisms in aggregate.Amyotrophic lateral sclerosis-associated mutant SOD1 inhibits anterograde axonal transport of mitochondria by reducing Miro1 levels.Current Advances and Limitations in Modeling ALS/FTD in a Dish Using Induced Pluripotent Stem Cells.C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation.Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.Inhibition of Drp1/Fis1 interaction slows progression of amyotrophic lateral sclerosis.Repeat-associated non-AUG (RAN) translation and other molecular mechanisms in Fragile X Tremor Ataxia Syndrome.Metabolic Dysregulation in Amyotrophic Lateral Sclerosis: Challenges and Opportunities.Representing Diversity in the Dish: Using Patient-Derived in Vitro Models to Recreate the Heterogeneity of Neurological Disease.The DNA damage response (DDR) is induced by the C9orf72 repeat expansion in amyotrophic lateral sclerosis.Genome-wide Analyses Identify KIF5A as a Novel ALS Gene.CRISPR-Cas9 screens in human cells and primary neurons identify modifiers of C9ORF72 dipeptide-repeat-protein toxicity.Perturbed autophagy and DNA repair converge to promote neurodegeneration in amyotrophic lateral sclerosis and dementia.Energy metabolism in ALS: an underappreciated opportunity?Repeat-Associated Non-ATG (RAN) Translation in Fuchs' Endothelial Corneal Dystrophy.A novel CHCHD10 mutation implicates a Mia40-dependent mitochondrial import deficit in ALS.Interactome analyses revealed that the U1 snRNP machinery overlaps extensively with the RNAP II machinery and contains multiple ALS/SMA-causative proteins.Amyotrophic lateral sclerosisThe proline-arginine repeat protein linked to C9-ALS/FTD causes neuronal toxicity by inhibiting the DEAD-box RNA helicase-mediated ribosome biogenesisCharacterization of membrane penetration and cytotoxicity of C9orf72-encoding arginine-rich dipeptides
P2860
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P2860
Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.
description
2016 nî lūn-bûn
@nan
2016年の論文
@ja
2016年論文
@yue
2016年論文
@zh-hant
2016年論文
@zh-hk
2016年論文
@zh-mo
2016年論文
@zh-tw
2016年论文
@wuu
2016年论文
@zh
2016年论文
@zh-cn
name
Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.
@en
type
label
Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.
@en
prefLabel
Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.
@en
P2093
P2860
P1433
P1476
Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.
@en
P2093
Anna Karydas
Dejun Yang
Fen-Biao Gao
Helene Tran
Rodrigo Lopez-Gonzalez
Sandra Almeida
Tania F Gendron
P2860
P304
P356
10.1016/J.NEURON.2016.09.015
P407
P577
2016-09-20T00:00:00Z