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The DOI matches its Crossref record; 3 of the 3 references it cites were found in a registry or an index.

Loss of Huntingtin-Mediated BDNF Gene Transcription in Huntington's Disease

Zuccato, Chiara, Ciammola, Andrea, Rigamonti, Dorotea, Leavitt, Blair R., Goffredo, Donato, Conti, Luciano, MacDonald, Marcy E., Friedlander, Robert M., Silani, Vincenzo, Hayden, Michael R., Timmusk, Tõnis, Sipione, Simonetta, Cattaneo, Elena·2001·Science
DOI 10.1126/science.1059581
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Abstract

Huntingtin is a 350-kilodalton protein of unknown function that is mutated in Huntington's disease (HD), a neurodegenerative disorder. The mutant protein is presumed to acquire a toxic gain of function that is detrimental to striatal neurons in the brain. However, loss of a beneficial activity of wild-type huntingtin may also cause the death of striatal neurons. Here we demonstrate that wild-type huntingtin up-regulates transcription of brain-derived neurotrophic factor (BDNF), a pro-survival factor produced by cortical neurons that is necessary for survival of striatal neurons in the brain. We show that this beneficial activity of huntingtin is lost when the protein becomes mutated, resulting in decreased production of cortical BDNF. This leads to insufficient neurotrophic support for striatal neurons, which then die. Restoring wild-type huntingtin activity and increasing BDNF production may be therapeutic approaches for treating HD.

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Crossref · 10.1126/science.1059581

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Research works printed but not linked: 35

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Metadata

Authors
Zuccato, C., Ciammola, A., Rigamonti, D., Leavitt, B. R., Goffredo, D., Conti, L., MacDonald, M. E., Friedlander, R. M., Silani, V., Hayden, M. R., Timmusk, T., Sipione, S., & Cattaneo, E.
Affiliations
  • Department of Pharmacological Sciences, University of Milano, Via Balzaretti 9, 20133 Milano, Italy.
  • Centre for Molecular Medicine and Therapeutic Department of Medical Genetics, University of British Columbia, Vancouver, British Columbia, Canada.
  • Molecular Neurogenetics Unit, Massachusetts General Hospital, Charlestown, MA 02129, USA.
  • Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, MA 02115, USA.
  • Center of Excellence on Neurodegenerative Diseases, University of Milano, Italy.
  • Program of Molecular Neuroscience, Institute of Biotechnology, Helsinki, Finland, and Department of Developmental Neuroscience, Biomedical Center, Uppsala University, Uppsala, Sweden.
Venue
Science
Publisher
American Association for the Advancement of Science (AAAS)
Published
2001-07-20
Volume
293
Issue
5529
Pages
493-498
ISSN
  • 0036-8075
  • 1095-9203
DOI
10.1126/science.1059581
Cited by
1,123
References
3
Language
en

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