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Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease

Science, 21 June 2002
Vol. 296, Issue 5576, p. 2238-2243
DOI: 10.1126/science.1072613

Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease

  1. Anthone W. Dunah1,
  2. Hyunkyung Jeong1,
  3. April Griffin1,
  4. Yong-Man Kim2,
  5. David G. Standaert1,
  6. Steven M. Hersch1,
  7. M. Maral Mouradian2,
  8. Anne B. Young1,
  9. Naoko Tanese3,
  10. Dimitri Krainc1,*
  1. 1 Department of Neurology, Massachusetts General Hospital, Harvard Medical School, Center for Aging, Genetics and Neurodegeneration, Charlestown, MA 02129, USA.
  2. 2 Genetic Pharmacology Unit, Experimental Therapeutics Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, 10 Center Drive, MSC 1406, Bethesda, MD 20892–1406, USA.
  3. 3 Department of Microbiology/MSB258, New York University School of Medicine, 550 First Avenue, New York, NY 10016, USA.


Huntington's disease (HD) is an inherited neurodegenerative disease caused by expansion of a polyglutamine tract in the huntingtin protein. Transcriptional dysregulation has been implicated in HD pathogenesis. Here, we report that huntingtin interacts with the transcriptional activator Sp1 and coactivator TAFII130. Coexpression of Sp1 and TAFII130 in cultured striatal cells from wild-type and HD transgenic mice reverses the transcriptional inhibition of the dopamine D2 receptor gene caused by mutant huntingtin, as well as protects neurons from huntingtin-induced cellular toxicity. Furthermore, soluble mutant huntingtin inhibits Sp1 binding to DNA in postmortem brain tissues of both presymptomatic and affected HD patients. Understanding these early molecular events in HD may provide an opportunity to interfere with the effects of mutant huntingtin before the development of disease symptoms.

  • * To whom correspondence should be addressed. E-mail: krainc{at}

  • Received for publication 27 February 2002.
  • Accepted for publication 23 April 2002.


A. W. Dunah, H. Jeong, A. Griffin, Y.-M. Kim, D. G. Standaert, S. M. Hersch, M. M. Mouradian, A. B. Young, N. Tanese, and D. Krainc, Sp1 and TAFII130 Transcriptional Activity Disrupted in Early Huntington's Disease. Science 296, 2238-2243 (2002).

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