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. 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. Lusa 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 Dglon 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
