Glutamine Repeats and Neurodegenerative Diseases: Molecular AspectsPeter S. Harper, Max F. Perutz This book focuses on the discovery of a common genetic basis for a group of inherited neurological disorders, including Huntington's Disease, spino-bulbar atrophy and a series of hereditary ataxias. This shared molecular background and other similarities have led to the development of theoretical models for the pathogenesis of these diseases. It is now also clear that the mechanisms involved are likely to be of more general relevance, outside of this particular group of disorders, with implications for other neurodegenerative processes such as those involved in Alzheimer's, Parkinson's and Prion diseases. The book is an edited and updated compilation evolving from a Royal Society discussion meeting. |
Contents
a clinical genetic and molecular model | 1 |
A transgenic mouse model of Huntingtons disease | 13 |
Behavioural changes and selective neuronal loss | 41 |
Copyright | |
17 other sections not shown
Common terms and phrases
a-synuclein abnormal alleles Alzheimer's disease androgen receptor antibody apoptosis Aronin ataxin-1 atrophin-1 Bates bulbar muscular atrophy caspase cell death cellular cerebellar clinical cortex cytoplasmic Davies degeneration detected DiFiglia DRPLA dystrophic neurites exon expanded polyglutamine protein figure filaments Fischbeck formation fragment fusion proteins glutamine glutamine repeats glutamine residues Goedert Hackam HD gene human huntingtin Huntington's disease immunoreactivity interactions isoforms Lewy bodies loci Mandel Mangiarini microsatellites molecular mouse model mutant huntingtin mutations N-terminal Nature Genet nerve cells neurites neurodegenerative diseases neuronal neuronal intranuclear inclusions neuronal loss neuropathological NIIs normal nuclear inclusions nuclear localization nucleus onset pathogenesis pathology Paulson phenotype polyglutamine expansion polyglutamine tract repeat disorders repeat length Ross SBMA SCA1 SCA6 SCA7 Scherzinger sequence spinal and bulbar spinocerebellar ataxia spinocerebellar ataxia type staining striatal striatum studies subcellular tau isoforms tau protein toxicity transgenic mice transgenic mouse trinucleotide repeat Trottier truncated Turmaine ubiquitin vitro Vonsattel Wanker wild-type Zoghbi