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Book Reviews
June 2002

Glutamine Repeats and Neurodegenerative Diseases: Molecular Aspects

Author Affiliations


Arch Neurol. 2002;59(6):1038. doi:

edited by Peter S. Harper and Max Perutz, ISBN 0-19-850685-6, Oxford University Press Inc, New York, NY, 2001.

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 of the book, deals with tauopathies and α-synucleinopathies.