Huntington's Disease (4th edition)

ISBN : 9780199929146

Gillian Bates; Sarah Tabrizi; Lesley Jones
512 Pages
183 x 261 mm
Pub date
May 2014
Oxford Monographs on Medical Genetics
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This fourth edition of Huntington's Disease presents a comprehensive summary of the current knowledge of this disease, including the major scientific and clinical advances that have occurred since publication of the third edition in 2002. Completely updated and expanded, chapters in this volume are organized in five sections: * Clinical aspects of Huntington's disease, including updated chapters on historical perspectives, neurological, neuropsychiatric, and neuropsychological aspects, and new chapters on juvenile Huntington's and the premanifest and early stages * The genetics of Huntington's disease, including new information on its epidemiology discussions of new testing guidelines * Neurobiology, including recent insights into correlations between pathology and symptoms and a new chapter on neuronal circuitry * The molecular biology of Huntington's disease, including new chapters on the normal function of huntingtin, the molecular pathogenesis of Huntington's disease and the peripheral pathology of the disorder, and an extensively updated chapter on its structural biology * An updated description of the comprehensive care for Huntington's disease, featureing a new chapter on preclinical therapeutics and a completely rewritten chapter on the state of the art of experimental therapeutics and clinical trials.


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

About the author: 

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.

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