Polyglutamine Disorders

1. Clinical Features of Huntington’s Disease
Rhia Ghosh, Sarah J. Tabrizi

2. Genetic Rodent Models of Huntington Disease
J. Stricker-Shaver, A. Novati, L. Yu-Taeger, H. P. Nguyen

3. Mitochondrial Dysfunction in Huntington’s Disease
Catarina Carmo, Luana Naia, Carla Lopes, A. Cristina Rego

4. RNA Related Pathology in Huntington’s Disease
Andreas Neueder, Gillian P. Bates

5. X-Linked Spinal and Bulbar Muscular Atrophy: From Clinical Genetic Features and Molecular Pathology to Mechanisms Underlying Disease Toxicity
Constanza J. Cortes, Albert R. Spada

6. Spinocerebellar Ataxia Type 1: Molecular Mechanisms of Neurodegeneration and Preclinical Studies
Judit M. Pérez Ortiz, Harry T. Orr

7. Spinocerebellum Ataxia Type 6: Molecular Mechanisms and Calcium Channel Genetics
Xiaofei Du, Christopher Manuel Gomez

8. Spinocerebellar Ataxia Type 2
Daniel R. Scoles, Stefan M. Pulst

9. Molecular Mechanisms and Therapeutic Strategies in Spinocerebellar Ataxia Type 7
Alice Karam, Yvon Trottier

10. Spinocerebellar Ataxia Type 17 (SCA17)
Yasuko Toyoshima, Hitoshi Takahashi

11. The Neuropathology of Spinocerebellar Ataxia Type 3/Machado-Joseph Disease
Arnulf H. Koeppen

12. Origins and Spread of Machado-Joseph Disease Ancestral Mutations Events
Sandra Martins, Jorge Sequeiros

13. Clinical Features of Machado-Joseph Disease
Nuno Mendonça, Marcondes C. França, António Freire Gonçalves, Cristina Januário

14. Polyglutamine-Independent Features in Ataxin-3 Aggregation and Pathogenesis of Machado-Joseph Disease
Ana Luisa Carvalho, Alexandra Silva, Sandra Macedo-Ribeiro

15. Animal Models of Machado-Joseph Disease
Jana Schmidt, Thorsten Schmidt

16. Towards the Identification of Molecular Biomarkers of Spinocerebellar Ataxia Type 3 (SCA3)/Machado-Joseph Disease (MJD)
Manuela Lima, Mafalda Raposo

17. Planning Future Clinical Trials for Machado-Joseph Disease
Jonas Alex Morales Saute, Laura Bannach Jardim

18. Molecular Mechanisms and Cellular Pathways Implicated in Machado-Joseph Disease Pathogenesis

Clévio Nóbrega, Ana Teresa Simões, Joana Duarte-Neves, Sónia Duarte, Ana Vasconcelos-Ferreira, Janete Cunha-Santos, Dina Pereira, Magda Santana, Cláudia Cavadas, Luís Pereira Almeida

19. Pharmacological Therapies for Machado-Joseph Disease
Sara Duarte-Silva, Patrícia Maciel

20. Gene Therapies for Polyglutamine Diseases
Carlos A. Matos, Vítor Carmona, Udaya-Geetha Vijayakumar, Sara Lopes, Patrícia Albuquerque, Mariana Conceição, Rui Jorge Nobre, Clévio Nóbrega, Luís Pereira Almeida

21. Stem Cell-Based Therapies for Polyglutamine Diseases
Liliana S. Mendonça, Isabel Onofre, Catarina Oliveira Miranda, Rita Perfeito, Clévio Nóbrega, Luís Pereira Almeida

Keywords: Biomedicine, Neurosciences, Neurology, Gene Function