Huntington’s Disease

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. 

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. Luísa 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 Déglon

 

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