Niels Skotte

Niels Skotte

Visiting Professor


  1. 2017
  2. Published

    A novel humanized mouse model of Huntington disease for preclinical development of therapeutics targeting mutant huntingtin alleles

    Southwell, A. L., Skotte, Niels Henning, Villanueva, Erika Bianca, Østergaard, M. E., Gu, X., Kordasiewicz, H. B., Kay, C., Cheung, D., Xie, Y., Waltl, S., Dal Cengio, L., Findlay-Black, H., Doty, C. N., Petoukhov, E., Iworima, D., Slama, R., Ooi, J., Pouladi, M. A., Yang, X. W., Swayze, E. E., Seth, P. P. & Hayden, M. R., 15 Mar 2017, In: Human Molecular Genetics. 26, 6, p. 1115-1132 18 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Palmitoylation of caspase-6 by HIP14 regulates its activation

    Skotte, Niels Henning, Sanders, S. S., Singaraja, R. R., Ehrnhoefer, D. E., Vaid, K., Qiu, X., Kannan, S., Verma, C. & Hayden, M. R., 2017, In: Cell Death and Differentiation. 24, p. 433-444 12 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  4. 2016
  5. Interactome network analysis identifies multiple caspase-6 interactors involved in the pathogenesis of HD

    Riechers, S., Butland, S., Deng, Y., Skotte, Niels Henning, Ehrnhoefer, D. E., Russ, J., Laine, J., Laroche, M., Pouladi, M. A., Wanker, E. E., Hayden, M. R. & Graham, R. K., 15 Apr 2016, In: Human Molecular Genetics. 25, 8, p. 1600-18 19 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  6. 2015
  7. Huntingtin Haplotypes Provide Prioritized Target Panels for Allele-specific Silencing in Huntington Disease Patients of European Ancestry

    Kay, C., Collins, J. A., Skotte, Niels Henning, Southwell, A. L., Warby, S. C., Caron, N. S., Doty, C. N., Nguyen, B., Griguoli, A., Ross, C. J., Squitieri, F. & Hayden, M. R., Nov 2015, In: Molecular Therapy. 23, 11, p. 1759-71 13 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  8. 2014
  9. In vivo evaluation of candidate allele-specific mutant huntingtin gene silencing antisense oligonucleotides

    Southwell, A. L., Skotte, Niels Henning, Kordasiewicz, H. B., Østergaard, M. E., Watt, A. T., Carroll, J. B., Doty, C. N., Villanueva, Erika Bianca, Petoukhov, E., Vaid, K., Xie, Y., Freier, S. M., Swayze, E. E., Seth, P. P., Bennett, C. F. & Hayden, M. R., Dec 2014, In: Molecular Therapy. 22, 12, p. 2093-106 14 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  10. Personalized gene silencing therapeutics for Huntington disease

    Kay, C., Skotte, Niels Henning, Southwell, A. L. & Hayden, M. R., Jul 2014, In: Clinical Genetics. 86, 1, p. 29-36 8 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  11. HACE1 reduces oxidative stress and mutant Huntingtin toxicity by promoting the NRF2 response

    Rotblat, B., Southwell, A. L., Ehrnhoefer, D. E., Skotte, Niels Henning, Metzler, M., Franciosi, S., Leprivier, G., Somasekharan, S. P., Barokas, A., Deng, Y., Tang, T., Mathers, J., Cetinbas, N., Daugaard, M., Kwok, B., Li, L., Carnie, C. J., Fink, D., Nitsch, R., Galpin, J. D., Ahern, C. A., Melino, G., Penninger, J. M., Hayden, M. R. & Sorensen, P. H., 25 Feb 2014, In: Proceedings of the National Academy of Sciences of the United States of America. 111, 8, p. 3032-7 6 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  12. Published

    p53 increases caspase-6 expression and activation in muscle tissue expressing mutant huntingtin

    Ehrnhoefer, D. E., Skotte, Niels Henning, Ladha, S., Nguyen, Y. T. N., Qiu, X., Deng, Y., Huynh, K. T., Engemann, S., Nielsen, S. M., Becanovic, K., Leavitt, B. R., Hasholt, Lis Frydenreich & Hayden, M. R., 1 Feb 2014, In: Human Molecular Genetics. 23, 3, p. 717-29 13 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

  13. Allele-specific suppression of mutant huntingtin using antisense oligonucleotides: providing a therapeutic option for all Huntington disease patients

    Skotte, Niels Henning, Southwell, A. L., Østergaard, M. E., Carroll, J. B., Warby, S. C., Doty, C. N., Petoukhov, E., Vaid, K., Kordasiewicz, H., Watt, A. T., Freier, S. M., Hung, G., Seth, P. P., Bennett, C. F., Swayze, E. E. & Hayden, M. R., 2014, In: PLOS ONE. 9, 9, p. e107434

    Research output: Contribution to journalJournal articleResearchpeer-review

  14. 2013
  15. Rational design of antisense oligonucleotides targeting single nucleotide polymorphisms for potent and allele selective suppression of mutant Huntingtin in the CNS

    Østergaard, M. E., Southwell, A. L., Kordasiewicz, H., Watt, A. T., Skotte, Niels Henning, Doty, C. N., Vaid, K., Villanueva, Erika Bianca, Swayze, E. E., Bennett, C. F., Hayden, M. R. & Seth, P. P., Nov 2013, In: Nucleic Acids Research. 41, 21, p. 9634-50 17 p.

    Research output: Contribution to journalJournal articleResearchpeer-review

ID: 7791