Huntington's Disease

Professor Gillian Bates obtained her PhD from the University of London in 1987. Her postdoctoral work was conducted with Hans Lehrach at the ICRF on the cloning of the Huntington's disease gene. Her work has at the forefront of Huntington's disease research since establishing her independent research programme in 1994. Professor Lesley Jones obtained her PhD from Cardiff University in 1990 and moved to the then University of Wales College of Medicine in 1994 to work on the biochemistry of huntingtin in HD. Her work has primarily been concerned with the notable downstream effects of mutant huntingtin on gene expression. She is also leading work on genetic modifiers of HD to assist in the identification of molecular pathways important in disease pathology. Sarah Tabrizi is Professor of Clinical Neurology in the Department of Neurodegenerative Diseases at the UCL Institute of Neurology and Honorary Consultant Neurologist at the National Hospital of Neurology and Neurosurgery. Her research focuses on the pathobiological, translational and clinical aspects of neurodegeneration with a major focus on Huntington's disease. Sarah leads two major, international multidisciplinary research initiatives, TRACK-HD and Track-On HD, aimed at understanding the neurobiology of the neurodegenerative changes in premanifest and early stage HD gene carriers and identifying sensitive measures of disease progression.

Table of Contents ; Section 1: Clinical aspects of Huntington's disease ; 1. Huntington's Disease in a Historical Context ; Peter S Harper ; 2. Clinical Neurology of Huntington's disease ; Raymund AC Roos ; 3. Neuropsychiatry and Neuropsychology ; David Craufurd and Julie Snowden ; 4. Juvenile Huntington's disease ; Oliver W J Quarrell ; 5. Premanifest and Early Huntington's Disease ; Edward J Wild and Sarah J Tabrizi ; Section 2: The genetics of Huntington's disease ; 6. Huntington's disease: genetic and molecular studies ; Cecile Cazeneuve and Alexandra Durr ; 7. The Epidemiology of Huntington's Disease ; Chris Kay, Emily Fisher, Michael R. Hayden ; 8. Genetic Counselling and Testing ; Rhona MacLeod and Aad Tibben ; Section 3: Neurobiology ; 9. The Neuropathology of Huntington's Disease in the Human Brain ; Henry J Waldvogel, Eric H Kim, Lynette J Tippett, Jean-Paul G Vonsattel, and Richard LM Faull ; 10. Altered Neuronal Circuitry in Huntington's Disease ; Michael S. Levine, Elizabeth A. Wang, Jane Y. Chen, Carlos Cepeda, and Veronique M. Andre ; Section 4: The molecular biology of Huntington's disease ; 11. The normal function of huntingtin ; Chiara Zuccato and Elena Cattaneo ; 12. Order, disorder, and conformational flux in the structural biology of Huntington's Disease ; Ronald Wetzel and Rakesh Mishra ; 13. Pathogenic mechanisms in Huntington's disease ; Alis Hughes and Lesley Jones ; 14. Peripheral pathology in Huntington's disease ; Authors: Jorien M.M. van der Burg, N. Ahmad Aziz, Maria Bjorkqvist ; Section 5: Therapeutic interventions in Huntington's disease ; 15. Comprehensive care in Huntington's disease ; Martha A. Nance ; 16. Preclinical Experimental Therapeutics in Huntington's Disease ; Gillian P. Bates and Christian Landles ; 17. Experimental Therapeutics in Huntington's Disease: Moving Forward in Clinical Trials ; Beth Borowsky and Cristina Sampaio