Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH

James R. Burke, Jan J. Enghild, Margaret E. Martin, Yuh Shan Jou, Richard M. Myers, Allen D. Roses, Jeffery M. Vance, Warren J. Strittmatter

Research output: Contribution to journalArticlepeer-review

400 Scopus citations


At least five adult-onset neurodegenerative diseases, including Huntington disease (HD), and dentatorubral-pallidoluysian atrophy (DRPLA) are produced by genes containing a variably increased CAG repeat within the coding region 1-4. The size range of the repeats is similar in all diseases; unaffected individuals have fewer than 30 CAG repeats, whereas affected patients usually have more than 40 repeats. The size of the inherited CAG repeat correlates with the severity and age of disease onset1,5-7. The CAG triplet repeat produces a polyglutamine domain in the expressed proteins3,8-10. All of these diseases are inherited in a dominant fashion, and a pathologic gain of function in gene carriers has been proposed. We sought to identify proteins in the brain that selectively interact with polyglutamine-domain proteins, hypothesizing that the polyglutamine domain may determine protein-protein interactions.

Original languageEnglish (US)
Pages (from-to)347-350
Number of pages4
JournalNature medicine
Issue number3
StatePublished - 1996
Externally publishedYes

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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