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Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis

Breakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS... Full description

Journal Title: Neuron 2013-08-07, Vol.79 (3), p.416-438
Main Author: Ling, Shuo-Chien
Other Authors: Polymenidou, Magdalini , Cleveland, Don W
Format: Electronic Article Electronic Article
Language: English
Subjects:
RNA
Quelle: Alma/SFX Local Collection
Publisher: United States: Elsevier Inc
ID: ISSN: 0896-6273
Link: https://www.ncbi.nlm.nih.gov/pubmed/23931993
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recordid: cdi_pubmedcentral_primary_oai_pubmedcentral_nih_gov_4411085
title: Converging Mechanisms in ALS and FTD: Disrupted RNA and Protein Homeostasis
format: Article
creator:
  • Ling, Shuo-Chien
  • Polymenidou, Magdalini
  • Cleveland, Don W
subjects:
  • Adenosine Triphosphatases - genetics
  • Adenosine Triphosphatases - metabolism
  • Alzheimers disease
  • Amyotrophic lateral sclerosis
  • Amyotrophic Lateral Sclerosis - genetics
  • Analysis
  • Animals
  • Article
  • Autophagy
  • C9orf72 Protein
  • Cell Cycle Proteins - genetics
  • Cell Cycle Proteins - metabolism
  • Dementia
  • Development and progression
  • DNA-Binding Proteins - genetics
  • DNA-Binding Proteins - metabolism
  • Frontotemporal Dementia - genetics
  • Genes
  • Genetic research
  • Homeostasis
  • Homeostasis - genetics
  • Humans
  • Metabolism
  • Models, Biological
  • Mutation
  • Mutation - genetics
  • Neurons
  • Neuroscience(all)
  • Pathogenesis
  • Proteins
  • Proteins - genetics
  • Proteins - metabolism
  • RNA
  • RNA - metabolism
  • RNA-Binding Protein FUS - genetics
  • RNA-Binding Protein FUS - metabolism
  • Rodents
  • Ubiquitins - genetics
  • Ubiquitins - metabolism
  • Valosin Containing Protein
ispartof: Neuron, 2013-08-07, Vol.79 (3), p.416-438
description: Breakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression. In this Review, Ling, Polymenidou, and Cleveland propose that paired dysfunction in RNA processing and protein homeostasis in amyotrophic lateral sclerosis and frontotemporal dementia establishes a feedforward loop involving cell-to-cell prion-like spread that drives disease progression.
language: eng
source: Alma/SFX Local Collection
identifier: ISSN: 0896-6273
fulltext: fulltext
issn:
  • 0896-6273
  • 1097-4199
url: Link


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descriptionBreakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression. In this Review, Ling, Polymenidou, and Cleveland propose that paired dysfunction in RNA processing and protein homeostasis in amyotrophic lateral sclerosis and frontotemporal dementia establishes a feedforward loop involving cell-to-cell prion-like spread that drives disease progression.
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subjectAdenosine Triphosphatases - genetics ; Adenosine Triphosphatases - metabolism ; Alzheimers disease ; Amyotrophic lateral sclerosis ; Amyotrophic Lateral Sclerosis - genetics ; Analysis ; Animals ; Article ; Autophagy ; C9orf72 Protein ; Cell Cycle Proteins - genetics ; Cell Cycle Proteins - metabolism ; Dementia ; Development and progression ; DNA-Binding Proteins - genetics ; DNA-Binding Proteins - metabolism ; Frontotemporal Dementia - genetics ; Genes ; Genetic research ; Homeostasis ; Homeostasis - genetics ; Humans ; Metabolism ; Models, Biological ; Mutation ; Mutation - genetics ; Neurons ; Neuroscience(all) ; Pathogenesis ; Proteins ; Proteins - genetics ; Proteins - metabolism ; RNA ; RNA - metabolism ; RNA-Binding Protein FUS - genetics ; RNA-Binding Protein FUS - metabolism ; Rodents ; Ubiquitins - genetics ; Ubiquitins - metabolism ; Valosin Containing Protein
ispartofNeuron, 2013-08-07, Vol.79 (3), p.416-438
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descriptionBreakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression. In this Review, Ling, Polymenidou, and Cleveland propose that paired dysfunction in RNA processing and protein homeostasis in amyotrophic lateral sclerosis and frontotemporal dementia establishes a feedforward loop involving cell-to-cell prion-like spread that drives disease progression.
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abstractBreakthrough discoveries identifying common genetic causes for amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these disorders. They share unexpectedly similar signatures, including dysregulation in common molecular players including TDP-43, FUS/TLS, ubiquilin-2, VCP, and expanded hexanucleotide repeats within the C9ORF72 gene. Dysfunction in RNA processing and protein homeostasis is an emerging theme. We present the case here that these two processes are intimately linked, with disease-initiated perturbation of either leading to further deviation of both protein and RNA homeostasis through a feedforward loop including cell-to-cell prion-like spread that may represent the mechanism for relentless disease progression. In this Review, Ling, Polymenidou, and Cleveland propose that paired dysfunction in RNA processing and protein homeostasis in amyotrophic lateral sclerosis and frontotemporal dementia establishes a feedforward loop involving cell-to-cell prion-like spread that drives disease progression.
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