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Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.

Publication ,  Journal Article
Burke, JR; Enghild, JJ; Martin, ME; Jou, YS; Myers, RM; Roses, AD; Vance, JM; Strittmatter, WJ
Published in: Nat Med
March 1996
Published version (DOI) Link to item

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.

Duke Scholars

James Robert Burke
Author James Robert Burke Neurology, Behavioral Neurology
Warren James Strittmatter
Author Warren James Strittmatter Neurology, Behavioral Neurology
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Published In

Nat Med

DOI

10.1038/nm0396-347

ISSN

1078-8956

Publication Date

March 1996

Volume

2

Issue

3

Start / End Page

347 / 350

Location

United States

Related Subject Headings

  • Trinucleotide Repeats
  • Rabbits
  • Protein Binding
  • Nuclear Proteins
  • Nervous System Diseases
  • Nerve Tissue Proteins
  • Molecular Sequence Data
  • Minisatellite Repeats
  • In Vitro Techniques
  • Immunology
 

Citation

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Burke, J. R., Enghild, J. J., Martin, M. E., Jou, Y. S., Myers, R. M., Roses, A. D., … Strittmatter, W. J. (1996). Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med, 2(3), 347–350. https://doi.org/10.1038/nm0396-347
Burke, J. R., J. J. Enghild, M. E. Martin, Y. S. Jou, R. M. Myers, A. D. Roses, J. M. Vance, and W. J. Strittmatter. “Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.” Nat Med 2, no. 3 (March 1996): 347–50. https://doi.org/10.1038/nm0396-347.
Burke JR, Enghild JJ, Martin ME, Jou YS, Myers RM, Roses AD, et al. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med. 1996 Mar;2(3):347–50.
Burke, J. R., et al. “Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH.” Nat Med, vol. 2, no. 3, Mar. 1996, pp. 347–50. Pubmed, doi:10.1038/nm0396-347.
Burke JR, Enghild JJ, Martin ME, Jou YS, Myers RM, Roses AD, Vance JM, Strittmatter WJ. Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med. 1996 Mar;2(3):347–350.

Published In

Nat Med

DOI

10.1038/nm0396-347

ISSN

1078-8956

Publication Date

March 1996

Volume

2

Issue

3

Start / End Page

347 / 350

Location

United States

Related Subject Headings

  • Trinucleotide Repeats
  • Rabbits
  • Protein Binding
  • Nuclear Proteins
  • Nervous System Diseases
  • Nerve Tissue Proteins
  • Molecular Sequence Data
  • Minisatellite Repeats
  • In Vitro Techniques
  • Immunology