Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.


Journal Article

At least five adult-onset neurodegenerative diseases, including Huntingtin disease (HD), and dentatorubral-pallidoluysian atrophy (DRPLA) are produced by genes containing a variably increased CAG repeat within the coding region. 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 onset. The CAG triplet repeat produces a polyglutamine domain in the expressed proteins. 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.

Full Text

Duke Authors

Cited Authors

  • Burke, JR; Enghild, JJ; Martin, ME; Jou, YS; Myers, RM; Roses, AD; Vance, JM; Strittmatter, WJ

Published Date

  • March 1996

Published In

Volume / Issue

  • 2 / 3

Start / End Page

  • 347 - 350

PubMed ID

  • 8612237

Pubmed Central ID

  • 8612237

International Standard Serial Number (ISSN)

  • 1078-8956

Digital Object Identifier (DOI)

  • 10.1038/nm0396-347


  • eng

Conference Location

  • United States