C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
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Bidirectional nucleolar dysfunction in C9orf72 frontotemporal lobar degenerationProtein Quality Control and the Amyotrophic Lateral Sclerosis/Frontotemporal Dementia ContinuumMouse Models of C9orf72 Hexanucleotide Repeat Expansion in Amyotrophic Lateral Sclerosis/ Frontotemporal DementiaRNA stores tau reversibly in complex coacervatesGranulostasis: Protein Quality Control of RNP Granules.Dysregulation of RNA Binding Protein Aggregation in Neurodegenerative Disorders.TDP-43 and FUS en route from the nucleus to the cytoplasm.Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 geneThe Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the ProteasomeToxic PRn poly-dipeptides encoded by the C9orf72 repeat expansion block nuclear import and export.Drosophila melanogaster As a Model Organism to Study RNA Toxicity of Repeat Expansion-Associated Neurodegenerative and Neuromuscular DiseasesMyosin II is not required for Drosophila tracheal branch elongation and cell intercalation.Evidence that C9ORF72 Dipeptide Repeat Proteins Associate with U2 snRNP to Cause Mis-splicing in ALS/FTD Patients.The small heat shock protein B8 (HSPB8) efficiently removes aggregating species of dipeptides produced in C9ORF72-related neurodegenerative diseases.Viral delivery of C9orf72 hexanucleotide repeat expansions in mice leads to repeat-length-dependent neuropathology and behavioural deficits.Integrating the genomic architecture of human nucleolar organizer regions with the biophysical properties of nucleoli.c9orf72 Disease-Related Foci Are Each Composed of One Mutant Expanded Repeat RNA.Fluid-Based Biomarkers for Amyotrophic Lateral Sclerosis.The nucleus: keeping it together by keeping it apart.The Role of Dipeptide Repeats in C9ORF72-Related ALS-FTD.Synaptic Vesicle Clusters at Synapses: A Distinct Liquid Phase?New developments in RAN translation: insights from multiple diseases.Control of mRNA Translation in ALS ProteinopathyRNA-binding proteins with prion-like domains in health and disease.Interactions of pathological proteins in neurodegenerative diseases.Energy Homeostasis and Abnormal RNA Metabolism in Amyotrophic Lateral SclerosisRNA Misprocessing in C9orf72-Linked Neurodegeneration.Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.Failure to Deliver and Translate-New Insights into RNA Dysregulation in ALS.RNA biology of disease-associated microsatellite repeat expansions.Proteostasis disturbance in amyotrophic lateral sclerosis.Genetic mutations in RNA-binding proteins and their roles in ALS.A PR plug for the nuclear pore in amyotrophic lateral sclerosis.Phosphorylation of the FUS low-complexity domain disrupts phase separation, aggregation, and toxicity.Ribosomal stress and Tp53-mediated neuronal apoptosis in response to capsid protein of the Zika virus.Nuclear localized C9orf72-associated arginine-containing dipeptides exhibit age-dependent toxicity in C. elegans.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.Regulation of mRNA Translation in Neurons-A Matter of Life and Death.Intrinsically disordered linkers determine the interplay between phase separation and gelation in multivalent proteins.TDP-43 pathology disrupts nuclear pore complexes and nucleocytoplasmic transport in ALS/FTD.
P2860
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P2860
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
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
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@en
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@nl
type
label
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@en
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@nl
prefLabel
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@en
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@nl
P2093
P2860
P50
P1433
P1476
C9orf72 Dipeptide Repeats Impa ...... n of Membrane-Less Organelles.
@en
P2093
Amandine Molliex
Brian D Freibaum
Diana M Mitrea
Hong Joo Kim
Jaclyn Cika
James Messing
Jamshid Temirov
Jennifer Moore
Junmin Peng
P2860
P304
774-788.e17
P356
10.1016/J.CELL.2016.10.002
P407
P577
2016-10-01T00:00:00Z