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Molecular Mechanisms Involved in the Pathogenesis of Huntington's Disease
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Authors: Claudia Perandones, Federico Eduardo Micheli and Martín Radrizzani (University of Buenos Aires, Argentina, and others) 
Book Description:
Huntington's disease (HD) is an autosomal-dominant, progressive neurodegenerative disorder with a distinct phenotype, including chorea, incoordination, cognitive decline, and behavioral difficulties. The underlying genetic defect responsible for the disease is the expansion of a CAG repeat in the gene coding for the HD protein, huntingtin (htt). This CAG repeat is an unstable triplet repeat DNA sequence, and its length is inversely correlated with the age at onset of the disease. Expanded CAG repeats have been found in 8 other inherited neurodegenerative diseases. Despite its widespread distribution, mutant htt causes selective neurodegeneration, which occurs preferentially and most prominently in the striatum and deeper layers of the cortex. This book focuses on HD, outlining the effects of mutant htt in the nucleus and cytoplasm as well as the role of cell-cell interactions in the HD pathology.
(Imprint: Nova Biomedical)

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Table of Contents:
1.Huntington`s Disease pp.1-22

1.1.Introduction: Unstable expanding repeats as a novel cause of

1.2. Common features of diseases due to unstable expanding repeats

1.3. Clinical and genetic aspects of HD

1.4. Epidemiology of HD

1.5. Clinical Correlates in HD

1.6. Isolation of the HD mutation

1.7. The HD trinucleotide repeat mutation1

1.8.Neuropathologic studies in HD

2. Molecular pathogenesis of Huntington`s Disease pp.23-36

2.1. HD gene and Gene Product

2.2. Correlation between neuropathology and pathogenesis in HD

2.3. Models for the pathogenesis of HD

2.4. Nuclear effect of mutant huntingtin

2.5. Cytoplasmic effect of mutant huntingtin

3. Conclusion pp.37-38

References pp.39-50

Index pp.51-56

      Neurodegenerative Diseases - Laboratory and Clinical Research
   Binding: Softcover
   Pub. Date: 2010
   Pages: 56.pp
   ISBN: 978-1-61728-971-2
   Status: AV
Status Code Description
AN Announcing
FM Formatting
PP Page Proofs
FP Final Production
EP Editorial Production
PR At Prepress
AP At Press
AV Available
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Molecular Mechanisms Involved in the Pathogenesis of Huntington's Disease