Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons. - Wikidata - awgldk - TriplyDB

Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.

Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.

http://www.wikidata.org/entity/Q38739863

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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 vivo Pathogenic 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 Sclerosis Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.The Enigmatic Role of C9ORF72 in Autophagy Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.Axonal Degeneration during Aging and Its Functional Role in Neurodegenerative Disorders Autophagy 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 sclerosis The proline-arginine repeat protein linked to C9-ALS/FTD causes neuronal toxicity by inhibiting the DEAD-box RNA helicase-mediated ribosome biogenesis Characterization of membrane penetration and cytotoxicity of C9orf72-encoding arginine-rich dipeptides

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Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.

http://www.wikidata.org/entity/Q38739863

description

2016 nî lūn-bûn

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2016年论文

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name

Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.

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Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.

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Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.

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J.NEURON.2016.09.015

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Poly(GR) in C9ORF72-Related AL ...... in iPSC-Derived Motor Neurons.

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Anna Karydas

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Dejun Yang

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Fen-Biao Gao

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Helene Tran

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Rodrigo Lopez-Gonzalez

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Sandra Almeida

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Tania F Gendron

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Yubing Lu

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P2860

RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention

Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS

Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS

A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD

Age-dependent deterioration of nuclear pore complexes causes a loss of nuclear integrity in postmitotic cells

The C9orf72 repeat expansion disrupts nucleocytoplasmic transport.

Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons

Regulation of p53 in response to DNA damage.

Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion

RNA-protein interactions in unstable microsatellite diseases.

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scholarly article

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10.1016/J.NEURON.2016.09.015

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2

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92

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Bruce L. Miller

Leonard Petrucelli

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2016-09-20T00:00:00Z

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27720481

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amyotrophic lateral sclerosis

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5111366

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