Huntingtin's critical cleavage

Article metrics

The early pathogenic events leading to neurodegeneration in Huntington disease are not clear. A recent paper shows that mutating a caspase-6 cleavage site in the huntingtin protein is sufficient to prevent pathogenesis.

Access optionsAccess options

Rent or Buy article

Get time limited or full article access on ReadCube.

from$8.99

All prices are NET prices.

Figure 1: Effects of mutating the caspase-6 cleavage site on the function and toxicity of polyglutamine-expanded htt.

Katie Ris

References

  1. 1

    Graham, R.K. et al. Cell 125, 1179–1191 (2006).

  2. 2

    Gatchel, J.R. & Zoghbi, H.Y. Nat. Rev. Genet. 6, 743–755 (2005).

  3. 3

    Ikeda, H. et al. Nat. Genet. 13, 196–202 (1996).

  4. 4

    Emamian, E.S. et al. Neuron 38, 375–387 (2003).

  5. 5

    Steffan, J.S. et al. Science 304, 100–104 (2004).

  6. 6

    Takeyama, K. et al. Neuron 35, 855–864 (2002).

  7. 7

    Tsuda, H. et al. Cell 122, 633–644 (2005).

  8. 8

    Kim, Y.J. et al. Proc. Natl. Acad. Sci. USA 98, 12784–12789 (2001).

  9. 9

    Graham, R.K. et al. Neurobiol. Dis. 21, 444–455 (2006).

  10. 10

    Van Raamsdonk, J.M. et al. Hum. Mol. Genet. 14, 3823–3835 (2005).

  11. 11

    Klement, I.A. et al. Cell 95, 41–53 (1998).

  12. 12

    Saudou, F., Finkbeiner, S., Devys, D. & Greenberg, M.E. Cell 95, 55–66 (1998).

  13. 13

    Hodgson, J.G. et al. Hum. Mol. Genet. 5, 1875–1885 (1996).

  14. 14

    Leavitt, B.R. et al. J. Neurochem. 96, 1121–1129 (2006).

  15. 15

    Goehler, H. et al. Mol. Cell 15, 853–865 (2004).

Download references

Author information

Correspondence to Huda Y Zoghbi.

Rights and permissions

Reprints and Permissions

About this article

Further reading