Niels Skotte

Niels Skotte

Assistant Professor, Visiting professor


  1. 2023
  2. 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 journalJournal articlepeer-review

  3. Accepted/In press
  4. 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 journalJournal articlepeer-review

  5. 2022
  6. 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 journalConference abstract in journalpeer-review

  7. 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 journalJournal articlepeer-review

  8. 2021
  9. Published

    Hippocampal disruptions of synaptic and astrocyte metabolism are primary events of early amyloid pathology in the 5xFAD mouse model of Alzheimer's disease

    Andersen, Jens Velde, Skotte, Niels Henning, Christensen, S. K., Polli, F. S., Shabani, M., Markussen, K. H., Haukedal, H., Westi, Emil Winther, Diaz-delCastillo, M., Sun, R. C., Kohlmeier, Kristi Anne, Schousboe, Arne, Gentry, M. S., Tanila, H., Freude, Kristine, Aldana, Blanca, Mann, Matthias & Waagepetersen, Helle S., 2021, In: Cell Death & Disease. 12, 11, 13 p., 954 .

    Research output: Contribution to journalJournal articlepeer-review

  10. 2020
  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 journalJournal articlepeer-review

  12. 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 journalJournal articlepeer-review

  13. 2019
  14. 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 journalJournal articlepeer-review

  15. Activation of Caspase-6 Is Promoted by a Mutant Huntingtin Fragment and Blocked by an Allosteric Inhibitor Compound

    Ehrnhoefer, D. E., Skotte, N. H., Reinshagen, J., Qiu, X., Windshuegel, B., Jaishankar, P., Ladha, S., Petina, O., Khankischpur, M., Nguyen, Y. T. N., Caron, N. S., Razeto, A., Zu Rheda, M. M., Deng, Y., Huynh, K. T., Wittig, I., Gribbon, P., Renslo, A. R., Geffken, D., Gul, S. & 1 others, Hayden, M. R., 2019, In: Cell Chemical Biology. 26, 9, p. 1295-1305.e6

    Research output: Contribution to journalJournal articlepeer-review

  16. Published

    Enhanced cerebral branched-chain amino acid metabolism in R6/2 mouse model of Huntington's disease

    Andersen, Jens Velde, Skotte, Niels Henning, Aldana, Blanca, Nørremølle, Anne & Waagepetersen, Helle S., 2019, In: Cellular and Molecular Life Sciences. 76, 12, p. 2449-2461

    Research output: Contribution to journalJournal articlepeer-review

  17. 2018
  18. 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 journalJournal articlepeer-review

  19. 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 journalJournal articlepeer-review

  20. Published

    Site-specific characterization of endogenous SUMOylation across species and organs

    Hendriks, Ivo Alexander, Lyon, D., Su, D., Skotte, Niels Henning, Daniel, J. A., Jensen, Lars Juhl & Nielsen, Michael Lund, 2018, In: Nature Communications. 9, p. 1-17 2456.

    Research output: Contribution to journalJournal articlepeer-review

  21. 2017
  22. 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 journalJournal articlepeer-review

  23. 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 articlepeer-review

  24. 2016
  25. 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 articlepeer-review

  26. 2015
  27. 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 articlepeer-review

  28. 2014
  29. 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 articlepeer-review

  30. 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 articlepeer-review

  31. 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 journalJournal articlepeer-review

  32. 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 articlepeer-review

  33. 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 articlepeer-review

  34. 2013
  35. 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 articlepeer-review

  36. 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 journalJournal articlepeer-review

  37. 2012
  38. 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 journalJournal articlepeer-review

  39. 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 journalJournal articlepeer-review

  40. 2011
  41. 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 journalJournal articlepeer-review

  42. Published

    Potent and selective antisense oligonucleotides targeting single-nucleotide polymorphisms in the Huntington disease gene / allele-specific silencing of mutant huntingtin

    Carroll, J. B., Warby, S. C., Southwell, A. L., Doty, C. N., Greenlee, S., Skotte, Niels Henning, Hung, G., Bennett, C. F., Freier, S. M. & Hayden, M. R., Dec 2011, In: Molecular therapy : the journal of the American Society of Gene Therapy. 19, 12, p. 2178-85 8 p.

    Research output: Contribution to journalJournal articlepeer-review

  43. Published
  44. Published

    A quantitative method for the specific assessment of caspase-6 activity in cell culture

    Ehrnhoefer, D. E., Skotte, Niels Henning, Savill, J., Nguyen, Y. T. N., Ladha, S., Cao, L., Dullaghan, E. & Hayden, M. R., 2011, In: PLOS ONE. 6, 11, p. e27680

    Research output: Contribution to journalJournal articlepeer-review

  45. 2010
  46. 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 journalJournal articlepeer-review

  47. 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 journalJournal articlepeer-review

ID: 7791