Huntington's disease / [edited by] Gillian Bates, Peter S. Harper, Lesley Jones.

Format:

Book

Contributor:

Bates, Gillian.

Harper, Peter S.

Jones, Lesley, 1957-

Series:

Oxford monographs on medical genetics ; 45.

Oxford monographs on medical genetics ; 45

Language:

English

Subjects (All):

Huntington's disease.

Huntington Disease.

Medical Subjects:

Huntington Disease.

Physical Description:

xvi, 558 pages : illustrations, maps, portraits ; 25 cm.

Edition:

Third edition.

Place of Publication:

Oxford ; New York : Oxford University Press, 2002.

Summary:

It is now almost a decade since the identification of the Huntington's Disease gene and its mutation. Major advances in our understanding of the disorder have been made during this time. Since publication of the first two editions of this book, considerable progress has been made into understanding how the mutation causes the symptoms and brain pathology of HD. Experimental tools are in place to take this research further towards new therapeutic approaches. Due to these major advances, this well-established book has required radical updating. An international group of researchers and clinicians with specialist interests in Huntington's Disease have been commissioned to document the major recent advances in the genetics and neurobiology of the disease. Developments in our understanding of how the molecular basis of the disorder results in brain degeneration, with full coverage of transgenic animal models, neurochemical studies and advances in neuropathology are discussed in detail. The clinical sections cover both neurological and psychiatric aspects as well as new developments in therapy. This book will continue to provide an invaluable source of information for clinicians and scientists involved with Huntington's Disease, including geneticists, psychiatrists, neurologists and basic scientists in genetics and neurobiology.

Contents:

Section 1 Clinical aspects of Huntington's disease

1 Huntington's disease: a historical background / Peter S. Harper 3

2 Clinical neurology of Huntington's disease / Berry Kremer 28

3 Neuropsychological and neuropsychiatric aspects of Huntington's disease / David Craufurd, Julie Snowden 62

4 Imaging Huntington's disease / David J. Brooks, Thomasin Andrews 95

Section 2 The genetics of Huntington's disease

5 Huntington's disease: genetic and molecular studies / Peter S. Harper, Lesley Jones 113

6 The epidemiology of Huntington's disease / Peter S. Harper 159

7 Genetic counselling and presymptomatic testing / Aad Tibben 198

Section 3 Neurobiology

8 The neuropathology of Huntington's disease / Claire-Anne Gutekunst, Francine Norflus, Steven M. Hersch 251

9 Neurochemistry of Huntington's disease / George J. Yohrling, IV, Jang-Ho J. Cha 276

10 Energy metabolism and Huntington's disease / C. Turner, A.H.V. Schapira 309

Section 4 Molecular biology of Huntington's disease

11 Structural biology of Huntington's disease / Erich E. Wanker, Anja Droge 327

12 The cell biology of Huntington's disease / Lesley Jones 348

13 Mouse models of Huntington's disease / Gillian P. Bates, Kerry P.S.J. Murphy 387

Section 5 Other polyglutamine diseases

14 The polyglutamine diseases / Gillian P. Bates, Caroline Benn 429

Section 6 Therapeutic interventions

15 Comprehensive care in Huntington's disease / Martha A. Nance, Beryl Westphal 475

16 Therapeutic trials in Huntington's disease / Karl Kieburtz, Ira Shoulson 501

17 Cell and tissue transplantation / Stephen B. Dunnett, Anne E. Rosser 512.

Notes:

Previous ed.: London : W.B. Saunders, 1996.

Includes bibliographical references and index.

ISBN:

0198510608

OCLC:

51000460