The monoclonal antibody 3B5H10 that recognizes diffuse mutant huntingtin predicts cell death in primary striatal neurons. Biochemical and biophysical analyses reveal that the 3B5H10 epitope is exposed in a monomeric state, identifying this specific conformation as a toxic agent in Huntington's disease.
References
Orr, H.T. & Zoghbi, H.Y. Annu. Rev. Neurosci. 30, 575–621 (2007).
Bates, G. Lancet 361, 1642–1644 (2003).
Saudou, F., Finkbeiner, S., Devys, D. & Greenberg, M.E. Cell 95, 55–66 (1998).
Arrasate, M., Mitra, S., Schweitzer, E.S., Segal, M.R. & Finkbeiner, S. Nature 431, 805–810 (2004).
Haass, C. & Selkoe, D.J. Nat. Rev. Mol. Cell Biol. 8, 101–112 (2007).
Miller, J. et al. Nat. Chem. Biol. 7, 925–934 (2011).
Kayed, R. et al. Science 300, 486–489 (2003).
Paramithiotis, E. et al. Nat. Med. 9, 893–899 (2003).
Arrasate, M. & Finkbeiner, S. Proc. Natl. Acad. Sci. USA 102, 3840–3845 (2005).
Nekooki-Machida, Y. et al. Proc. Natl. Acad. Sci. USA 106, 9679–9684 (2009).
Author information
Authors and Affiliations
Corresponding author
Ethics declarations
Competing interests
The author declares no competing financial interests.
Rights and permissions
About this article
Cite this article
Tanaka, M. Tracking a toxic polyQ epitope. Nat Chem Biol 7, 861–862 (2011). https://doi.org/10.1038/nchembio.718
Published:
Issue Date:
DOI: https://doi.org/10.1038/nchembio.718
- Springer Nature America, Inc.