Open access peer-reviewed Edited Volume

Amyotrophic Lateral Sclerosis

Recent Advances and Therapeutic Challenges

Edited by Muralidhar L. Hegde

Houston Methodist Research Institute

A flurry of recent research on the role of the RNA/DNA-binding proteins TDP-43 and FUS as well as a dozen other factors (e.g., C9ORF72 and profilin) has led to a new paradigm in our understanding of the pathobiology of the motor neuron disease, Amyotrophic Lateral Sclerosis (ALS). How these factors trigger neuromuscular dysfunction is critical for developing more effective ALS therapeutics. The ‘gain-of-toxicity’ or ‘loss-of-function’ of these etiological factors is a key question. Recent studies on the imbalance in genome damage versus repair have opened avenues for potential DNA repair-based therapeutics. This book highlights emerging science in the area of ALS and discusses key approaches and mechanisms essential for developing a cure for ALS.

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Amyotrophic Lateral SclerosisRecent Advances and Therapeutic ChallengesEdited by Muralidhar L. Hegde

Published: August 26th 2020

DOI: 10.5772/intechopen.78121

ISBN: 978-1-83880-581-4

Print ISBN: 978-1-83880-580-7

eBook (PDF) ISBN: 978-1-83880-582-1

Copyright year: 2020

Books open for chapter submissions

833 Total Chapter Downloads

chaptersDownloads

Open access peer-reviewed

1. Molecular Basis of DNA Repair Defects in FUS-Associated ALS: Implications of a New Paradigm and Its Potential as Therapeutic Target

By Haibo Wang and Muralidhar L. Hegde

77

Open access peer-reviewed

2. Senataxin: A Putative RNA: DNA Helicase Mutated in ALS4—Emerging Mechanisms of Genome Stability in Motor Neurons

By Arijit Dutta, Robert Hromas and Patrick Sung

126

Open access peer-reviewed

3. Pathological Interaction between DNA Repair and Mitochondrial Dysfunction in ALS

By Luis Bermúdez-Guzmán and Alejandro Leal

172

Open access peer-reviewed

4. The Role of TDP-43 in Genome Repair and beyond in Amyotrophic Lateral Sclerosis

By Joy Mitra and Muralidhar L. Hegde

69

Open access peer-reviewed

5. The Extraocular Muscles Are Selectively Spared in ALS

By Fatima Pedrosa Domellöf

89

Open access peer-reviewed

6. The Role of Extracellular Vesicles in the Progression of ALS and Their Potential as Biomarkers and Therapeutic Agents with Which to Combat the Disease

By Changho Chun, Alec S.T. Smith, Mark Bothwell and David L. Mack

88

Open access peer-reviewed

7. RNA Metabolism and Therapeutics in Amyotrophic Lateral Sclerosis

By Orietta Pansarasa, Stella Gagliardi, Daisy Sproviero and Cristina Cereda

131

Open access peer-reviewed

8. Targeting Axonal Transport: A New Therapeutic Avenue for ALS

By Wenting Guo, Laura Fumagalli and Ludo Van Den Bosch

81

Edited Volume and chapters are indexed in

  • Worldcat
  • OpenAIRE
  • Google Scholar
  • AZ ebsco
  • Base
  • CNKI

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