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Open access peer-reviewed Edited Volume

Amyotrophic Lateral Sclerosis

Edited by Martin Henrik Maurer

Heidelberg University

Though considerable amount of research, both pre-clinical and clinical, has been conducted during recent years, Amyotrophic Lateral Sclerosis (ALS) remains one of the mysterious diseases of the 21st century. Great efforts have been made to develop pathophysiological models and to clarify the underlying pathology, and with novel instruments in genetics and transgenic techniques, the aim for finding a durable cure comes into scope. On the other hand, most pharmacological trials failed to show a benefit for ALS patients. In this book, the reader will find a compilation of state-of-the-art reviews about the etiology, epidemiology, and pathophysiology of ALS, the molecular basis of disease progression and clinical manifestations, the genetics familial ALS, as well as novel diagnostic criteria in the field of electrophysiology. An overview over all relevant pharmacological trials in ALS patients is also included, while the book concludes with a discussion on current advances and future trends in ALS research.

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Amyotrophic Lateral SclerosisEdited by Martin Henrik Maurer

Published: January 20th 2012

DOI: 10.5772/1054

ISBN: 978-953-307-806-9

eBook (PDF) ISBN: 978-953-51-4379-6

Copyright year: 2012

Books open for chapter submissions

85952 Total Chapter Downloads

34 Crossref Citations

89 Web of Science Citations

113 Dimensions Citations


Open access peer-reviewed

1. Amyotrophic Lateral Sclerosis: An Introduction to Treatment and Trials

By Martin H. Maurer


Open access peer-reviewed

2. Insights Arising from Gene Expression Profiling in Amyotrophic Lateral Sclerosis

By Johnathan Cooper-Knock, Joanna J. Bury, Laura Ferraiuolo, Emily F. Goodall, Pamela J. Shaw and Janine Kirby


Open access peer-reviewed

3. Dynamic Meta-Analysis as a Therapeutic Prediction Tool for Amyotrophic Lateral Sclerosis

By Cassie S. Mitchell and Robert H. Lee


Open access peer-reviewed

4. In Vivo and In Vitro Models to Study Amyotrophic Lateral Sclerosis

By François Berthod and François Gros-Louis


Open access peer-reviewed

5. Advantages and Pitfalls in Experimental Models Of ALS

By Marina Boido, Elisa Buschini, Antonio Piras, Giada Spigolon, Valeria Valsecchi, Letizia Mazzini and Alessandro Vercelli


Open access peer-reviewed

6. Electrophysiological Abnormalities in SOD1 Transgenic Models in Amyotrophic Lateral Sclerosis: The Commonalities and Differences

By Sherif M. Elbasiouny, Katharina A. Quinlan, Tahra L. Eissa and Charles J. Heckman


Open access peer-reviewed

7. Molecular and Electrical Abnormalities in the Mouse Model of Amyotrophic Lateral Sclerosis

By Katharina A. Quinlan, Sherif M. Elbasiouny and C.J. Heckman


Open access peer-reviewed

8. Role of Mitochondrial Dysfunction in Motor Neuron Degeneration in ALS

By Luz Diana Santa-Cruz, Uri Nimrod Ramírez-Jarquín and Ricardo Tapia


Open access peer-reviewed

9. Role of Neuronal Mitochondrial Metabolic Phenotype in Pathogenesis of ALS

By Alexander Panov, Nury Steuerwald, Valentin Vavilin, Svetlana Dambinova and Herbert L. Bonkovsky


Open access peer-reviewed

10. Mutant Cu/Zn-Superoxide Dismutase Induced Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis

By Jari Koistinaho and Gundars Goldsteins


Open access peer-reviewed

11. Folding and Aggregation of Cu, Zn-Superoxide Dismutase

By Helen R. Broom, Heather A. Primmer, Jessica A.O. Rumfeldt, Peter B. Stathopulos, Kenrick A. Vassall, Young-Mi Hwang and Elizabeth M. Meiering


Open access peer-reviewed

12. Oxidative Modifications of Cu, Zn-Superoxide Dismutase (SOD1) – The Relevance to Amyotrophic Lateral Sclerosis (ALS)

By Seiichi Nagano


Open access peer-reviewed

13. Reactive Nitrogen Species in Motor Neuron Apoptosis

By María Clara Franco and Alvaro G. Estévez


Open access peer-reviewed

14. Protein Aggregates in Pathological Inclusions of Amyotrophic Lateral Sclerosis

By Yoshiaki Furukawa


Open access peer-reviewed

15. The Kynurenine Pathway

By Yiquan Chen and Gilles Guillemin


Open access peer-reviewed

16. The Astrocytic Contribution in ALS: Inflammation and Excitotoxicity

By Kim Staats and Ludo Van Den Bosch


Open access peer-reviewed

17. Innate Immunity in ALS

By John D. Lee, Jia Y. Lee, Stephen M. Taylor, Peter G. Noakes and Trent M. Woodruff


Open access peer-reviewed

18. The Role of TNF-Alpha in ALS: New Hypotheses for Future Therapeutic Approaches

By Cristina Cereda, Stella Gagliardi, Emanuela Cova, Luca Diamanti and Mauro Ceroni


Open access peer-reviewed

19. Stem Cell Application for Amyotrophic Lateral Sclerosis: Growth Factor Delivery and Cell Therapy

By Masatoshi Suzuki, Chak Foon Tso and Michael G. Meyer


Open access peer-reviewed

20. Glial Cells as Therapeutic Targets for ALS

By Amanda M. Haidet-Phillips and Nicholas J. Maragakis


Open access peer-reviewed

21. Genetics of Amyotrophic Lateral Sclerosis

By Max Koppers, Michael van Es, Leonard H. van den Berg, Jan H. Veldink and R. Jeroen Pasterkamp


Open access peer-reviewed

22. Genetics of Familial Amyotrophic Lateral Sclerosis

By Emily F. Goodall, Joanna J. Bury, Johnathan Cooper-Knock, Pamela J. Shaw and Janine Kirby


Open access peer-reviewed

23. A Major Genetic Factor at Chromosome 9p Implicated in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD)

By Ilse Gijselinck, Kristel Sleegers, Christine Van Broeckhoven and Marc Cruts


Open access peer-reviewed

24. Multidisciplinary Rehabilitation in Amyotrophic Lateral Sclerosis

By Louisa Ng and Fary Khan


Open access peer-reviewed

25. Assessment and Management of Respiratory Dysfunction in Patients with Amyotrophic Lateral Sclerosis

By Daniele Lo Coco, Paolo Volanti, Domenico De Cicco, Antonio Spanevello, Gianluca Battaglia, Santino Marchese, Alfonsa Claudia Taiello, Rossella Spataro and Vincenzo La Bella


Open access peer-reviewed

26. Nutritional Care in Amyotrophic Lateral Sclerosis: An Alternative for the Maximization of the Nutritional State

By Luciano Bruno de Carvalho-Silva


Open access peer-reviewed

27. How to Assess Disease’s Severity and Monitor Patients with Amyotrophic Lateral Sclerosis: Lessons from Neurophysiology

By Ferdinando Sartucci, Tommaso Bocci, Lucia Briscese, Chiara Pecori, Chiara Rossi and Fabio Giannini


Open access peer-reviewed

28. Protection of Motor Neurons in Pre-Symptomatic Individuals Carrying SOD 1 Mutations: Results of Motor Unit Number Estimation (MUNE) Electrophysiology

By Arun Aggarwal


Open access peer-reviewed

29. Communication Impairment in ALS Patients Assessment and Treatment

By Paolo Bongioanni


Open access peer-reviewed

30. Human Computer Interactions for Amyotrophic Lateral Sclerosis Patients

By Ali Bülent Uşaklı


Open access peer-reviewed

31. Overview of Cognitive Function in ALS, with Special Attention to the Temporal Lobe: Semantic Fluency and Rating the Approachability of Faces

By Heike Schmolck, Paul Schulz and Michele York


Edited Volume and chapters are indexed in

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

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