Trinucleotide repeats that expand in human disease form hairpin structures in vitro

A. Marquis Gacy, Geoffrey Goellner, Nenad Juranić, Slobodan Macura, Cynthia T. McMurray

Research output: Contribution to journalArticle

480 Scopus citations

Abstract

We show that repeating units from all reported disease genes are capable of forming hairpins of common structure and threshold stability. The threshold stability is roughly -50 kcal per hairpin and is influenced by the flanking sequence of the gene. Hairpin stability has two components, sequence and length; only DNA of select sequences and the correct length can form hairpins of threshold energy. There is a correlation among the ability to form hairpins of threshold stability, the sequence selectivity of expansion, and the length dependence of expansion. Additionally, hairpin formation provides a potential structural basis for the constancy of the CCG region of the Huntington's disease gene in individuals and explains the stabilizing effects of AGG interruptions in FMR1 alleles.

Original languageEnglish (US)
Pages (from-to)533-540
Number of pages8
JournalCell
Volume81
Issue number4
DOIs
StatePublished - May 19 1995

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)

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