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Glutamine Repeats and Neurodegenerative Diseases: Molecular Aspects
edited by Peter S. Harper and Max Perutz, ISBN 0-19-850685-6, Oxford
University Press Inc, New York, NY, 2001.
Arch Neurol. 2002;59:1038.
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The first disorder caused by trinucleotide repeat expansion—namely,
Kennedy syndrome—was discovered in 1991. Thus, it is fitting that a
decade later, a book reviewing the clinical genetic and pathologic features
of trinucleotide repeat disorders is published.
This book is edited by 2 internationally respected individuals known
for their work in neurogenetic disorders, with chapters written by scientists
who have made significant contributions in this area. After an introductory
chapter on Huntington disease (HD) by Dr Harper, subsequent chapters describe
in detail animal models of HD (both mouse and Drosophila), proposed mechanisms of polyglutamine pathogenesis, and the biochemistry
of huntington in cell culture and in vitro. A smaller
section is devoted to the mechanisms of CAG instability and evolution. The
last 6 chapters review the phenotype and genotype correlations in spinocerebellar
ataxias, dentatorubral-pallidoluysian atrophy, and Kennedy disease. In this
section, the last chapter, somewhat of a surprise considering the focus . . . [Full Text of this Article]
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