Amyotrophic Lateral Sclerosis Part 1

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Amyotrophic Lateral Sclerosis Part 1 AMYOTROPHIC LATERAL SCLEROSIS Edited by Martin H. Maurer Amyotrophic Lateral SclerosisEdited by Martin H. MaurerPublished by InTechJaneza Trdine 9, 51000 Rijeka, CroatiaCopyright © 2011 InTechAll chapters are Open Access distributed under the Creative Commons Attribution 3.0license, which allows users to download, copy and build upon published articles even forcommercial purposes, as long as the author and publisher are properly credited, whichensures maximum dissemination and a wider impact of our publications. After this workhas been published by InTech, authors have the right to republish it, in whole or part, inany publication of which they are the author, and to make other personal use of thework. Any republication, referencing or personal use of the work must explicitly identifythe original source.As for readers, this license allows users to download, copy and build upon publishedchapters even for commercial purposes, as long as the author and publisher are properlycredited, which ensures maximum dissemination and a wider impact of our publications.NoticeStatements and opinions expressed in the chapters are these of the individual contributorsand not necessarily those of the editors or publisher. No responsibility is accepted for theaccuracy of information contained in the published chapters. The publisher assumes noresponsibility for any damage or injury to persons or property arising out of the use of anymaterials, instructions, methods or ideas contained in the book.Publishing Process Manager Adriana PecarTechnical Editor Teodora SmiljanicCover Designer InTech Design TeamImage Copyright gubh83, 2011. DepositPhotosFirst published January, 2012Printed in CroatiaA free online edition of this book is available at www.intechopen.comAdditional hard copies can be obtained from orders@intechweb.orgAmyotrophic Lateral Sclerosis, Edited by Martin H. Maurer p. cm.978-953-307-806-9free online editions of InTechBooks and Journals can be found atwww.intechopen.comVI Contents Part 2 Signalling Pathways and Molecular Pathophysiology 195 Chapter 8 Role of Mitochondrial Dysfunction in Motor Neuron Degeneration in ALS 197 Luz Diana Santa-Cruz, Uri Nimrod Ramírez-Jarquín and Ricardo Tapia Chapter 9 Role of Neuronal Mitochondrial Metabolic Phenotype in Pathogenesis of ALS 225 Alexander Panov, Nury Steuerwald, Valentin Vavilin, Svetlana Dambinova and Herbert L. Bonkovsky Chapter 10 Mutant Cu/Zn-Superoxide Dismutase Induced Mitochondrial Dysfunction in Amyotrophic Lateral Sclerosis 249 Jari Koistinaho and Gundars Goldsteins Chapter 11 Folding and Aggregation of Cu, Zn-Superoxide Dismutase 265 Helen R. Broom, Heather A. Primmer, Jessica A.O. Rumfeldt, Peter B. Stathopulos, Kenrick A. Vassall, Young-Mi Hwang and Elizabeth M. Meiering Chapter 12 Oxidative Modifications of Cu, Zn-Superoxide Dismutase (SOD1) – The Relevance to Amyotrophic Lateral Sclerosis (ALS) 301 Seiichi Nagano Chapter 13 Reactive Nitrogen Species in Motor Neuron Apoptosis 313 María Clara Franco and Alvaro G. Estévez Chapter 14 Protein Aggregates in Pathological Inclusions of Amyotrophic Lateral Sclerosis 335 Yoshiaki Furukawa Chapter 15 The Kynurenine Pathway 357 Yiquan Chen and Gilles Guillemin Part 3 Cellular Pathophysiology, the Immune System and Stem Cell Strategies 375 Chapter 16 The Astrocytic Contribution in ALS: Inflammation and Excitotoxicity 377 Kim Staats and Ludo Van Den Bosch Chapter 17 Innate Immunity in ALS 393 John D. Lee, Jia Y. Lee, Stephen M. Taylor, Peter G. Noakes and Trent M. Woodruff Contents VIIChapter 18 The Role of TNF-Alpha in ALS: New Hypotheses for Future Therapeutic Approaches 413 Cristina Cereda, Stella Gagliardi, Emanuela Cova, Luca Diamanti and Mauro C ...