Main description:

This detailed book provides a laboratory manual and guidebook for the selection, implementation, and interpretation of a wide range of techniques in contemporary use in leading laboratories engaged in Huntington's disease (HD) research worldwide. Only by understanding the pathology and pathogenic process at the fundamental molecular and cellular level can the research community expect to be able to slow or halt the disease process, repair the damage, and develop novel effective therapies to treat the symptoms of this condition, thus this volume collects the practical knowledge of its authors. Written for the highly successful Methods in Molecular Biology series, chapters include introductions to their respective topics, lists of the necessary materials and reagents, step-by-step, readily reproducible laboratory protocols, and tips on troubleshooting and avoiding known pitfalls.
Authoritative and invaluable, Huntington's Disease aims to help scientists to significantly extend the breadth and quality of research in laboratories dedicated to mastering and controlling this devastating human condition.

Contents:

1. Stereological Methods to Quantify Cell Loss in the Huntington's Disease Human Brain

Nasim F. Mehrabi, Malvindar K. Singh-Bains, Henry J. Waldvogel, and Richard L.M. Faull

2. Assessing Autophagic Activity and Aggregate Formation of Mutant Huntingtin in Mammalian Cells

Eleanna Stamatakou, Ye Zhu, and David C. Rubinsztein

3. A Filter Retardation Assay Facilitates the Detection and Quantification of Heat-Stable, Amyloidogenic Mutant Huntingtin Aggregates in Complex Biosamples

Anne Steinhof, Franziska Schindler, Alexander Buntru, Sigrid Schnoegl, and Erich E. Wanker

4. Cellular Models: HD Patient-Derived Pluripotent Stem Cells

Charlene Geater, Sarah Hernandez, Leslie Thompson, and Virginia B. Mattis

5. Non-Mammalian Models of Huntington's Disease

Anjalika Chongtham, Brett Barbaro, Tomas Filip, Adeela Syed, Weijian Huang, Marianne R. Smith, and J. Lawrence Marsh

6. Mouse Models of Huntington's Disease

Pamela P. Farshim and Gillian P. Bates

7. Motor Assessment in Huntington's Disease Mice

Stephen B. Dunnett and Simon P. Brooks

8. Automated Operant Assessments of Huntington's Disease Mouse Models

Emma Yhnell and Andreas Heuer

9. Neurophysiological Assessment of Huntington's Disease Model Mice

Elissa J. Donzis, Sandra M. Holley, Carlos Cepeda, and Michael S. Levine

10. Murine Models of Huntington's Disease for Evaluating Therapeutics

Natalia Kosior and Blair R. Leavitt

11. Generating Excitotoxic Lesion Models of Huntington's Disease

Mariah J. Lelos and Stephen B. Dunnett

12. Large-Brained Animal Models of Huntington's Disease: Sheep

A. Jennifer Morton

13. Minipigs as Large-Brained Animal Model for Huntington's Disease: From Behavior and Imaging to Gene Therapy

Ralf Reilmann and Verena Schuldenzucker

14. Non-Human Primate Models of Huntington's Disease and Their Application in Translational Research

Romina Aron Badin

15. In Vivo Multidimensional Brain Imaging in Huntington's Disease Animal Models

Julien Flament, Philippe Hantraye, and Julien Valette

16. Magnetic Resonance Imaging in Huntington's Disease

Sarah Gregory, Rachael I. Scahill, Geraint Rees, and Sarah Tabrizi

17. Biofluid Biomarkers in Huntington's Disease

Filipe B. Rodrigues, Lauren M. Byrne, and Edward J. Wild

18. Assessing and Modulating Kynurenine Pathway Dynamics in Huntington's Disease: Focus on Kynurenine 3-Monooxygenase

Korrapati V. Sathyasaikumar, Carlo Breda, Robert Schwarcz, and Flaviano Giorgini

19. Assessing Mitochondrial Function in In Vitro and Ex Vivo Models of Huntington's Disease

I. Luisa Ferreira, Catarina Carmo, Luana Naia, Sandra Mota, and A. Cristina Rego

20. Using Genomic Data to Find Disease-Modifying Loci in Huntington's Disease (HD)

Peter Holmans and Tim Stone

21. CRISPR/Cas9-Mediated Genome Editing for Huntington's Disease

Gabriel Vachey and Nicole Deglon

22. Methods for Assessing DNA Repair and Repeat Expansion in Huntington's Disease

Thomas Massey, Branduff McAllister, and Lesley Jones

23. Translating Antisense Technology into a Treatment for Huntington's Disease

Roger M. Lane, Anne Smith, Tiffany Baumann, Marc Gleichmann, Dan Norris,

C. Frank Bennett, and Holly Kordasiewicz

24. Disease Modification through Trophic Factor Delivery

Mari Savolainen, Dwaine Emerich, and Jeffrey H. Kordower

25. Methods to Quantify Cell Signaling and GPCR Receptor Ligand Bias: Characterization of Drugs that Target the Endocannabinoid Receptors in Huntington's Disease

Amina M. Bagher, Robert B. Laprairie, Melanie E.M. Kelly, and Eileen M. Denovan-Wright

26. Dissection and Preparation of Human Primary Fetal Ganglionic Eminence Tissue for Research and Clinical Application

Victoria H. Roberton, Anne E. Rosser, Anne-Marie McGorrian, and Sophie V. Precious

27. Robust Induction of DARPP32-Expressing GABAergic Striatal Neurons from Human Pluripotent Stem Cells

Marija Fjodorova and Meng Li

28. Quality Assessment and Production of Human Cells for Clinical Use

Lindsay Fraser, Kevin Bruce, John M. Campbell, and Paul A. De Sousa