Huntington's DiseaseGillian Bates, Sarah Tabrizi, Lesley Jones 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. |
Contents
Section two Genetics of Huntingtons Disease | 107 |
Section three Neurobiology of Huntingtons Disease | 183 |
Section Four Molecular Biology of Huntingtons Disease | 241 |
Section five Therapeutic Interventions in Huntingtons Disease | 391 |
| 485 | |
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Common terms and phrases
abnormalities acid activity aggregation alleles altered amyloid analysis appear assessment associated atrophy behavioral binding Biol brain CAG repeat cause cell changes chapter chorea clinical cognitive common contribute correlation cortex cortical cytoplasmic decreased described diagnosis direct disorder dysfunction early ease effects et al evidence exon expansion expression factor Figure findings formation fragments function gene Genet HD patients human Huntington’s disease impairment important improved increased indicate individuals interactions involved later levels loss mechanisms mice molecular motor mouse model mutant Htt mutant huntingtin Neurology neurons Neurosci normal observed occur onset pathway patients performance phenotype polyglutamine polyQ population possible potential predictive present prevalence progression protein psychiatric range recent receptor reduced region repeat length reported result risk role sequence showed shown specific stage striatal striatum structure studies suggest symptoms testing tion toxicity transcription transgenic treatment trials