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9780198506850

Glutamine Repeats and Neurodegenerative Diseases Molecular Aspects

by ;
  • ISBN13:

    9780198506850

  • ISBN10:

    0198506856

  • Format: Hardcover
  • Copyright: 2001-02-22
  • Publisher: Oxford University Press
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Summary

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.

Table of Contents

Preface ix
List of contributors
xii
Huntington's disease: a clinical, genetic and molecular model for polyglutamine repeat disorders
01(12)
P. S. Harper
Animal models of Huntington's disease
A Transgenic mouse model of Huntington's disease
13(13)
L. Mangiarini
K. Sathasivian
A. Mahal
B. Woodman
M. Turmaine
S. W. Davies
G. P. Bates
From neuronal inclusions to neurodegeneration: neuropathological investigation of a transgenic mouse model of Huntington's disease
26(15)
S. W. Davies
M. Turmaine
B. A. Cozens
A. S. Raza
A. Mahal
L. Mangiarini
G. P. Bates
Behavioural changes and selective neuronal loss in full-length transgenic mouse models for Huntington's disease
41(24)
P. H. Reddy
V. Charles
W. O. Whetsell, Jr.
D. A. Tagle
Evidence for both the nucleus and cytoplasm as subcellular sites of pathogenesis in Huntington's Disease in cell culture and in transgenic mice expressing mutant huntingtin
65(18)
A. S. Hackam
J. G. Hodgson
R. Singaraja
T. Zhang
L. Gan
C. A. Gutekunst
S. M. Hersch
M. R. Hayden
A genetic model for human polyglutamine-repeat disease in Drosophila melanogaster
83(10)
N. M. Bonini
Polyglutamine toxicity
Polyglutamine pathogenesis, potential role of protein interactions, proteolytic processing and nuclear localization
93(20)
C. A. Ross
J. D. Wood
M. F. Peters
G. Schilling
F. C. Nucifora Jr.
J. K. Cooper
A. H. Sharp
R. L. Margolis
A. Sawa
D. R. Borchelt
Properties of polyglutamine expansion in vitro and in a cellular model for Huntington's disease
113(14)
A. Lunkes
Y. Trottier
J. Fagart
P. Schultz
G. Zeder-Lutz
D. Moras
J. L. Mandel
Evidence for a recruitment and sequestration mechanism in Huntington's disease
127(16)
E. Preisinger
B. M. Jordan
A. Kazantsev
D. Housman
Biochemistry of huntingtin in cell cultures and in vitro
Aggregation of truncated GST-HD exon 1 fusion proteins containing normal range and dexpanded glutamine repeats
143(9)
B. Hollenbach
E. Scherzinger
K. Schweiger
R. Lurz
H. Lehrach
E. E. Wanker
The localization and interactions of huntingtin
152(14)
A. L. Jones
Analysis of the subecellular localization of huntingtion with a set of rabbit polyclonal antibodies in cultured mammalian cells of neuronal origin: comparison with the distribution of huntingtin in Huntington's disease autopsy brain
166(13)
J. C. Dorsman
M. A. Smoor
M. L. C. Maat-Schieman
M. Bout
S. Siesling
S. G. van Duinen
J. J. G. M. Verschuuren
J. T. den Dunnen
R. A. C. Roos
G. J. B. van Ommen
Are there multiple pathways in the pathogenesis of Huntington's disease?
179(20)
N. Aronin
M. Kim
G. Laforet
M. DiFiglia
Genomic influences on CAG instability
CAG repeat instability, cryptic sequence variation and pathogenicity: evedence from different loci
199(11)
M. Frontali
A. Novelletto
G. Annesi
C. Jodice
Microsatellite and trinucleotide repeat evolution: evidence for mutational bias and different rates of evolution in different lineages
210(11)
D. C. Rubinsztein
B. Amos
G. Cooper
Pathology caused by other proteins with expanded glutamine repeats
Genotype-phenotype correlation in the spinocerebellar ataxias
221(16)
P. F. Worth
A. Brice
N. W. Wood
Insights from mice carrying X-linked CAG-polyglutamine-repeat mutations
237(12)
J. M. Ordway
J. A. Cearley
P. J. Detloff
Molecular pathology of dentatorubral-pallidoluysian atrophy
249(12)
Ichiro Kanazawa
Androgen receptor mutation in Kennedy's disease
261(7)
K. H. Fishbeck
A. Lieberman
C. K. Bailey
A. Abel
D. E. Merry
Progress in Pathogenesis studies of spinocerebellar ataxia type 1
268(4)
C. J. Cummings
H. T. Orr
H. Y. Zoghbi
Filamentous nerve cell inclusions in neurodegenerative diseases: tauopathies and α-synucleinopathies
272(35)
Michael Goedert
Index 307

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