**Preface XIII**



Contents VII

Chapter 18 **The Role of TNF-Alpha in ALS:** 

Masatoshi Suzuki,

**Part 4 Human Genetics in ALS 477**

Chapter 22 **Genetics of Familial** 

Janine Kirby

Cristina Cereda, Stella Gagliardi,

Chapter 19 **Stem Cell Application for Amyotrophic Lateral** 

Chak Foon Tso and Michael G. Meyer

Chapter 20 **Glial Cells as Therapeutic Targets for ALS 455**

Chapter 21 **Genetics of Amyotrophic Lateral Sclerosis 479** 

Jan H. Veldink and R. Jeroen Pasterkamp

**Amyotrophic Lateral Sclerosis 517**  Emily F. Goodall, Joanna J. Bury,

Chapter 23 **A Major Genetic Factor at Chromosome** 

Ilse Gijselinck, Kristel Sleegers,

**Part 5 Clinical Research in ALS 555** 

Chapter 24 **Multidisciplinary Rehabilitation in**

Vincenzo La Bella

Chapter 26 **Nutritional Care in Amyotrophic**

Louisa Ng and Fary Khan

Daniele Lo Coco, Paolo Volanti,

Johnathan Cooper-Knock, Pamela J. Shaw and

Christine Van Broeckhoven and Marc Cruts

**Amyotrophic Lateral Sclerosis 557** 

Domenico De Cicco, Antonio Spanevello, Gianluca Battaglia, Santino Marchese, Alfonsa Claudia Taiello, Rossella Spataro and

**Lateral Sclerosis: An Alternative for** 

Luciano Bruno de Carvalho-Silva

Chapter 25 **Assessment and Management of Respiratory Dysfunction in Patients with Amyotrophic Lateral Sclerosis 579** 

**the Maximization of the Nutritional State 595**

**New Hypotheses for Future Therapeutic Approaches 413**

**Sclerosis: Growth Factor Delivery and Cell Therapy 437**

Emanuela Cova, Luca Diamanti and Mauro Ceroni

Amanda M. Haidet-Phillips and Nicholas J. Maragakis

Max Koppers, Michael van Es, Leonard H. van den Berg,

**9p Implicated in Amyotrophic Lateral Sclerosis (ALS) and Frontotemporal Lobar Degeneration (FTLD) 537** 


Chapter 19 **Stem Cell Application for Amyotrophic Lateral Sclerosis: Growth Factor Delivery and Cell Therapy 437**  Masatoshi Suzuki, Chak Foon Tso and Michael G. Meyer

Chapter 20 **Glial Cells as Therapeutic Targets for ALS 455**  Amanda M. Haidet-Phillips and Nicholas J. Maragakis

### **Part 4 Human Genetics in ALS 477**

VI Contents

**Part 2 Signalling Pathways and Molecular Pathophysiology 195** 

**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

**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

**Zn-Superoxide Dismutase (SOD1) –** 

María Clara Franco and Alvaro G. Estévez

**Inflammation and Excitotoxicity 377**  Kim Staats and Ludo Van Den Bosch

Stephen M. Taylor, Peter G. Noakes and

**The Relevance to Amyotrophic Lateral Sclerosis (ALS) 301** 

**Pathological Inclusions of Amyotrophic Lateral Sclerosis 335** 

Chapter 13 **Reactive Nitrogen Species in Motor Neuron Apoptosis 313** 

**the Immune System and Stem Cell Strategies 375**

**in Motor Neuron Degeneration in ALS 197** 

Uri Nimrod Ramírez-Jarquín and Ricardo Tapia

Chapter 8 **Role of Mitochondrial Dysfunction**

Luz Diana Santa-Cruz,

Chapter 9 **Role of Neuronal Mitochondrial** 

Chapter 11 **Folding and Aggregation of** 

Elizabeth M. Meiering

Chapter 12 **Oxidative Modifications of Cu,** 

Seiichi Nagano

Chapter 14 **Protein Aggregates in** 

Yoshiaki Furukawa

Chapter 15 **The Kynurenine Pathway 357**

**Part 3 Cellular Pathophysiology,**

Chapter 17 **Innate Immunity in ALS 393**  John D. Lee, Jia Y. Lee,

Trent M. Woodruff

Chapter 16 **The Astrocytic Contribution in ALS:** 

Yiquan Chen and Gilles Guillemin

	- **Part 5 Clinical Research in ALS 555**


