Niels Skotte

Niels Skotte

Assistant Professor, Visiting professor

ORCID: 0000-0001-7027-7193

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  1. 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 journal › Journal article › Research › peer-review

  2. Published

    Dysregulated COMT Expression in Fragile X Syndrome

    Utami, K. H., Yusof, N. A. B. M., Garcia-Miralles, M., Skotte, Niels Henning, Nama, S., Sampath, P., Langley, S. R. & Pouladi, M. A., 2023, In: NeuroMolecular Medicine. 25, p. 644–649

    Research output: Contribution to journal › Journal article › Research › peer-review

  3. Published

    Integrative Analysis Identifies Key Molecular Signatures Underlying Neurodevelopmental Deficits in Fragile X Syndrome

    Utami, K. H., Skotte, Niels Henning, Colaço, A. R., Yusof, N. A. B. M., Sim, B., Yeo, X. Y., Bae, H., Garcia-Miralles, M., Radulescu, C. I., Chen, Q., Chaldaiopoulou, G., Liany, H., Nama, S., Peteri, U. A., Sampath, P., Castrén, M. L., Jung, S., Mann, Matthias & Pouladi, M. A., 2020, In: Biological Psychiatry. 88, 6, p. 500-511

    Research output: Contribution to journal › Journal article › Research › peer-review

  4. Rescue from excitotoxicity and axonal degeneration accompanied by age-dependent behavioral and neuroanatomical alterations in caspase-6-deficient mice

    Uribe, V., Wong, B. K. Y., Graham, R. K., Cusack, C. L., Skotte, N. H., Pouladi, M. A., Xie, Y., Feinberg, K., Ou, Y., Ouyang, Y., Deng, Y., Franciosi, S., Bissada, N., Spreeuw, A., Zhang, W., Ehrnhoefer, D. E., Vaid, K., Miller, F. D., Deshmukh, M., Howland, D. & 1 others, Hayden, M. R., 1 May 2012, In: Human Molecular Genetics. 21, 9, p. 1954-67 14 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  5. Published

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

    Southwell, A. L., Skotte, N. H., Villanueva, E. B., 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. & 2 others, Seth, P. P. & Hayden, M. R., 15 Mar 2017, In: Human Molecular Genetics. 26, 6, p. 1115-1132 18 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  6. A fully humanized transgenic mouse model of Huntington disease

    Southwell, A. L., Warby, S. C., Carroll, J. B., Doty, C. N., Skotte, Niels Henning, Zhang, W., Villanueva, Erika Bianca, Kovalik, V., Xie, Y., Pouladi, M. A., Collins, J. A., Yang, X. W., Franciosi, S. & Hayden, M. R., 1 Jan 2013, In: Human Molecular Genetics. 22, 1, p. 18-34 17 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  7. 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 journal › Journal article › Research › peer-review

  8. Huntingtin suppression restores cognitive function in a mouse model of Huntington's disease

    Southwell, A. L., Kordasiewicz, H. B., Langbehn, D., Skotte, Niels Henning, Parsons, M. P., Villanueva, Erika Bianca, Caron, N. S., Østergaard, M. E., Anderson, L. M., Xie, Y., Cengio, L. D., Findlay-Black, H., Doty, C. N., Fitsimmons, B., Swayze, E. E., Seth, P. P., Raymond, L. A., Frank Bennett, C. & Hayden, M. R., 2018, In: Science Translational Medicine. 10, 461, p. 1-12 eaar3959.

    Research output: Contribution to journal › Journal article › Research › peer-review

  9. Antisense oligonucleotide therapeutics for inherited neurodegenerative diseases

    Southwell, A. L., Skotte, Niels Henning, Bennett, C. F. & Hayden, M. R., Nov 2012, In: Trends in Molecular Medicine. 18, 11, p. 634-43 10 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  10. Published

    Modulation of mutant huntingtin expression and processing: Pathogenic pathways and novel therapeutic approaches in Huntington's disease

    Skotte, Niels Henning, Nov 2011

    Research output: Book/Report › Ph.D. thesis › Research

  11. Published

    Compromised IGF signaling causes caspase-6 activation in Huntington disease

    Skotte, Niels Henning, Pouladi, M. A., Ehrnhoefer, D. E., Huynh, K., Qiu, X., Nielsen, S. M. B., Nielsen, T. T., Nørremølle, Anne & Hayden, M. R., 2020, In: Experimental Neurology. 332, 113396.

    Research output: Contribution to journal › Journal article › Research › peer-review

  12. Published

    Integrative Characterization of the R6/2 Mouse Model of Huntington's Disease Reveals Dysfunctional Astrocyte Metabolism

    Skotte, Niels Henning, Andersen, Jens Velde, Santos, A., Aldana, Blanca, Willert, C. W., Nørremølle, Anne, Waagepetersen, Helle S. & Nielsen, Michael Lund, 2018, In: Cell Reports. 23, 7, p. 2211-2224 14 p.

    Research output: Contribution to journal › Journal article › Research › peer-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 journal › Journal article › Research › peer-review

  14. 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 journal › Journal article › Research › peer-review

  15. Polycystin-1: a key player in hereditary cystic kidney and liver disorders

    Skotte, Niels Henning, Dec 2011, In: Clinical Genetics. 80, 6, p. 507-9 3 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

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

    Rotblat, B., Southwell, A. L., Ehrnhoefer, D. E., Skotte, N. H., 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. & 5 others, 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 journal › Journal article › Research › peer-review

  17. 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 journal › Journal article › Research › peer-review

  18. Published

    D13 Saliva biomarker discovery in Huntington’s disease

    Qvist, Filippa Liliendahl, Hellem, M. N., Nielsen, Jørgen Erik, Mann, Matthias & Skotte, Niels Henning, 2022, In: Journal of Neurology, Neurosurgery and Psychiatry. 93, Suppl. 1, p. A24-A25

    Research output: Contribution to journal › Conference abstract in journal › Research › peer-review

  19. Published

    Full-length huntingtin levels modulate body weight by influencing insulin-like growth factor 1 expression

    Pouladi, M. A., Xie, Y., Skotte, Niels Henning, Ehrnhoefer, D. E., Graham, R. K., Kim, J. E., Bissada, N., Yang, X. W., Paganetti, P., Friedlander, R. M., Leavitt, B. R. & Hayden, M. R., 15 Apr 2010, In: Human Molecular Genetics. 19, 8, p. 1528-38 11 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  20. Published

    Functional Differences between Synaptic Mitochondria from the Striatum and the Cerebral Cortex

    Petersen, M. H., Willert, C. W., Andersen, Jens Velde, Waagepetersen, Helle S., Skotte, Niels Henning & Nørremølle, Anne, May 2019, In: Neuroscience. 406, p. 432-443 12 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

  21. Published

    Progressive Mitochondrial Dysfunction of Striatal Synapses in R6/2 Mouse Model of Huntington's Disease

    Petersen, M. H., Willert, C. W., Andersen, Jens Velde, Madsen, M., Waagepetersen, Helle S., Skotte, Niels Henning & Nørremølle, Anne, 2022, In: Journal of Huntington's disease. 11, 2, p. 121-140

    Research output: Contribution to journal › Journal article › Research › peer-review

  22. Published

    Characterising the RNA-binding protein atlas of the mammalian brain uncovers RBM5 misregulation in mouse models of Huntington's disease

    Mullari, M., Fossat, Nicolas Julien, Skotte, Niels Henning, Asenjo-Martinez, A., Humphreys, D. T., Bukh, Jens, Kirkeby, Agnete, Scheel, Troels Kasper Høyer & Nielsen, Michael Lund, 2023, In: Nature Communications. 14, 20 p., 4348.

    Research output: Contribution to journal › Journal article › Research › peer-review

  23. 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 journal › Journal article › Research › peer-review

  24. 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 journal › Journal article › Research › peer-review

  25. Published

    Reduced gluconeogenesis and lactate clearance in Huntington's disease

    Josefsen, K., Nielsen, S. M. B., Campos, A., Seifert, T., Hasholt, Lis Frydenreich, Nielsen, Jørgen Erik, Nørremølle, Anne, Skotte, Niels Henning, Secher, Niels H. & Quistorff, B., 1 Dec 2010, In: Neurobiology of Disease. 40, 3, p. 656-62 7 p.

    Research output: Contribution to journal › Journal article › Research › peer-review

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