Accessibility Skip to Global Navigation Skip to Local Navigation Skip to Content Skip to Search Skip to Site Map Menu

Associate Professor Stephanie Hughes

Dr Stephanie HughesAssociate Professor Stephanie Hughes is the Principal Investigator of the Neural Development and Disease laboratory in the Department of Biochemistry. The lab focuses on the development, maintenance and diseases associated with motor neurons.

Associate Professor Hughes' current research focuses on transcription factors of the forebrain embryonic zinc finger family and their role in neuronal development and maintenance, and on the molecular consequences of mutations in Batten disease, a childhood neurodegenerative disorder. The lab is also developing tools and gene therapy vectors for Batten disease as well as Alzheimer’s and Parkinson’s disease.

Read more about Associate Professor Hughes' research here.

^ Top of page

Publications

Cain, J. T., Likhite, S., White, K. A., Timm, D. J., Davis, S. S., Johnson, T. B., … Hughes, S. M., … Weimer, J. M. (2019). Gene therapy corrects brain and behavioral pathologies in CLN6-Batten disease. Molecular Therapy. Advance online publication. doi: 10.1016/j.ymthe.2019.06.015

Morrissey, J., Mockett, B. G., Singh, A., Kweon, D., Ohline, S. M., Tate, W. P., Hughes, S. M., & Abraham, W. C. (2019). A C-terminal peptide from secreted amyloid precursor protein-α enhances long-term potentiation in rats and a transgenic mouse model of Alzheimer's disease. Neuropharmacology. Advance online publication. doi: 10.1016/j.neuropharm.2019.107670

Morrissey, J. A., Bigus, E., Necarsulmer, J. C., Srinivasan, V., Peppercorn, K., O'Leary, D. J., Mockett, B. G., Tate, W. P., Hughes, S. M., … Abraham, W. C. (2019). The tripeptide RER mimics secreted amyloid precursor protein-alpha in upregulating LTP. Frontiers in Cellular Neuroscience, 13, 459. doi: 10.3389/fncel.2019.00459

Mathiesen, S. N., Ohline, S. M., Wicky, H. E., Cheong, M. Y. I., Abraham, W. C., & Hughes, S. M. (2019). Assessing the efficacy of a novel adeno-associated viral capsid in targeting the brain. New Zealand Medical Journal, 132(1499), (pp. 87). Retrieved from https://www.nzma.org.nz/journal

Ohline, S. M., Wake, K. L., Hawkridge, M.-V., Dinnunhan, M. F., Hegemann, R. U., Wilson, A., Schoderboeck, L., Logan, B. J., … Hughes, S. M., & Abraham, W. C. (2018). Adult-born dentate granule cell excitability depends on the interaction of neuron age, ontogenetic age and experience. Brain Structure & Function. Advance online publication. doi: 10.1007/s00429-018-1685-2

Chapter in Book - Research

Empson, R. M., Tantirigama, M. L. S., Oswald, M. J., Hughes, S. M., & Knöpfel, T. (2015). Combined immunochemistry and live imaging of fluorescent protein expressing neurons in mouse brain. In A. Merighi & L. Lossi (Eds.), Immunocytochemistry and related techniques. (pp. 357-373). New York: Springer. doi: 10.1007/978-1-4939-2313-7_19

^ Top of page

Journal - Research Article

Cain, J. T., Likhite, S., White, K. A., Timm, D. J., Davis, S. S., Johnson, T. B., … Hughes, S. M., … Weimer, J. M. (2019). Gene therapy corrects brain and behavioral pathologies in CLN6-Batten disease. Molecular Therapy. Advance online publication. doi: 10.1016/j.ymthe.2019.06.015

Morrissey, J. A., Bigus, E., Necarsulmer, J. C., Srinivasan, V., Peppercorn, K., O'Leary, D. J., Mockett, B. G., Tate, W. P., Hughes, S. M., … Abraham, W. C. (2019). The tripeptide RER mimics secreted amyloid precursor protein-alpha in upregulating LTP. Frontiers in Cellular Neuroscience, 13, 459. doi: 10.3389/fncel.2019.00459

Morrissey, J., Mockett, B. G., Singh, A., Kweon, D., Ohline, S. M., Tate, W. P., Hughes, S. M., & Abraham, W. C. (2019). A C-terminal peptide from secreted amyloid precursor protein-α enhances long-term potentiation in rats and a transgenic mouse model of Alzheimer's disease. Neuropharmacology. Advance online publication. doi: 10.1016/j.neuropharm.2019.107670

Ohline, S. M., Wake, K. L., Hawkridge, M.-V., Dinnunhan, M. F., Hegemann, R. U., Wilson, A., Schoderboeck, L., Logan, B. J., … Hughes, S. M., & Abraham, W. C. (2018). Adult-born dentate granule cell excitability depends on the interaction of neuron age, ontogenetic age and experience. Brain Structure & Function. Advance online publication. doi: 10.1007/s00429-018-1685-2

Mitchell, N. L., Russell, K. N., Wellby, M. P., Wicky, H. E., Schoderboeck, L., Barrell, G. K., Melzer, T. R., … Hughes, S. M., & Palmer, D. N. (2018). Longitudinal in vivo monitoring of CNS demonstrates the efficacy of gene therapy in a sheep model of CLN5 Batten disease. Molecular Therapy. Advance online publication. doi: 10.1016/j.ymthe.2018.07.015

Tan, V. T. Y., Mockett, B. G., Ohline, S. M., Parfitt, K. D., Wicky, H. E., Peppercorn, K., Schoderboeck, L., Yahaya, M. F. b., Tate, W. P., Hughes, S. M., & Abraham, W. C. (2018). Lentivirus-mediated expression of human secreted amyloid precursor protein-alpha prevents development of memory and plasticity deficits in a mouse model of Alzheimer's disease. Molecular Brain, 11, 7. doi: 10.1186/s13041-018-0348-9

Schoderboeck, L., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2018). Genetic targeting and chemogenetic inhibition of new born neurons. Human Gene Therapy Methods. Advance online publication. doi: 10.1089/hgtb.2018.182

Clare, A. J., Wicky, H. E., Empson, R. M., & Hughes, S. M. (2017). RNA-sequencing analysis reveals a regulatory role for transcription factor Fezf2 in the mature motor cortex. Frontiers in Molecular Neuroscience, 10, 283. doi: 10.3389/fnmol.2017.00283

Best, H. L., Neverman, N. J., Wicky, H. E., Mitchell, N. L., Leitch, B., & Hughes, S. M. (2017). Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics. Neurobiology of Disease, 100, 62-74. doi: 10.1016/j.nbd.2017.01.001

Sizemore, R. J., Seeger-Armbruster, S., Hughes, S. M., & Parr-Brownlie, L. C. (2016). Viral vector-based tools advance knowledge of basal ganglia anatomy and physiology. Journal of Neurophysiology, 115, 2124-2146. doi: 10.1152/jn.01131.2015

Tantirigama, M. L. S., Oswald, M. J., Clare, A. J., Wicky, H. E., Day, R. C., Hughes, S. M., & Empson, R. M. (2016). Fezf2 expression in layer 5 projection neurons of mature mouse motor cortex. Journal of Comparative Neurology, 524(4), 829-845. doi: 10.1002/cne.23875

Parr-Brownlie, L. C., Bosch-Bouju, C., Schoderboeck, L., Sizemore, R. J., Abraham, W. C., & Hughes, S. M. (2015). Lentiviral vectors as tools to understand central nervous system biology in mammalian model organisms. Frontiers in Molecular Neuroscience, 8, 14. doi: 10.3389/fnmol.2015.00014

Seeger-Armbruster, S., Bosch-Bouju, C., Little, S. T. C., Smither, R. A., Hughes, S. M., Hyland, B. I., & Parr-Brownlie, L. C. (2015). Patterned, but not tonic, optogenetic stimulation in motor thalamus improves reaching in acute drug-induced Parkinsonian rats. Journal of Neuroscience, 35(3), 1211-1216. doi: 10.1523/jneurosci.3277-14.2015

Schoderboeck, L., Riad, S., Bokor, A. M., Wicky, H. E., Strauss, M., Bostina, M., Oswald, M. J., Empson, R. M., & Hughes, S. M. (2015). Chimeric rabies SADB19-VSVg-pseudotyped lentiviral vectors mediate long-range retrograde transduction from the mouse spinal cord. Gene Therapy, 22, 357-364. doi: 10.1038/gt.2015.3

Neverman, N. J., Best, H. L., Hofmann, S. L., & Hughes, S. (2015). Experimental therapies in the neuronal ceroid lipofuscinoses. Biochimica et Biophysica Acta: Molecular Basis of Disease, 1852(10, Part B), 2292-2300. doi: 10.1016/j.bbadis.2015.04.026

Palmer, D. N., Neverman, N. J., Chen, J. Z., Chang, C.-T., Houweling, P. J., Barry, L. A., … Hughes, S. M., & Mitchell, N. L. (2015). Recent studies of ovine neuronal ceroid lipofuscinoses from BARN, the Batten Animal Research Network. Biochimica et Biophysica Acta: Molecular Basis of Disease, 1852(10, Part B), 2279-2286. doi: 10.1016/j.bbadis.2015.06.013

Hughes, S. M., Hope, K. M., Xu, J. B., Mitchell, N. L., & Palmer, D. N. (2014). Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy. Neurobiology of Disease, 62, 543-550. doi: 10.1016/j.nbd.2013.11.011

Tantirigama, M. L. S., Oswald, M. J., Duynstee, C., Hughes, S. M., & Empson, R. M. (2014). Expression of the developmental transcription factor Fezf2 identifies a distinct subpopulation of layer 5 intratelencephalic-projection neurons in mature mouse motor cortex. Journal of Neuroscience, 34(12), 4303-4308. doi: 10.1523/jneurosci.3111-13.2014

Oswald, M. J., Tantirigama, M. L. S., Sonntag, I., Hughes, S. M., & Empson, R. M. (2013). Diversity of layer 5 projection neurons in the mouse motor cortex. Frontiers in Cellular Neuroscience, 7, 174. doi: 10.3389/fncel.2013.00174

Jarvis, R. M., Hughes, S. M., & Ledgerwood, E. C. (2012). Peroxiredoxin 1 functions as a signal peroxidase to receive, transduce, and transmit peroxide signals in mammalian cells. Free Radical Biology & Medicine, 53(7), 1522-1530. doi: 10.1016/j.freeradbiomed.2012.08.001

Dottori, M., Tay, C., & Hughes, S. M. (2011). Neural development in human embryonic stem cells—Applications of lentiviral vectors. Journal of Cellular Biochemistry, 112(8), 1955-1962. doi: 10.1002/jcb.23116

Linterman, K. S., Palmer, D. N., Kay, G. W., Barry, L. A., Mitchell, N. L., McFarlane, R. G., Black, M. A., … Hughes, S. M. (2011). Lentiviral-mediated gene transfer to the sheep brain: Implications for gene therapy in Batten disease. Human Gene Therapy, 22(8), 1011-1020. doi: 10.1089/hum.2011.026

Kells, A. P., Henry, R. A., Hughes, S. M., & Connor, B. (2007). Verification of functional AAV-mediated neurotrophic and anti-apoptotic factor expression. Journal of Neuroscience Methods, 161(2), 291-300. doi: 10.1016/j.jneumeth.2006.11.006

Gibbons, H. M., Hughes, S. M., Van Roon-Mom, W., Greenwood, J. M., Narayan, P. J., Teoh, H. H., … Dragunow, M. (2007). Cellular composition of human glial cultures from adult biopsy brain tissue. Journal of Neuroscience Methods, 166(1), 89-98. doi: 10.1016/j.jneumeth.2007.07.005

Chen, K., Hughes, S. M., & Connor, B. (2007). Neural progenitor cells derived from the adult rat subventricular zone: Characterization and transplantation. Cell Transplantation, 16(8), 799-810.

Gordon, R. J., Tattersfield, A. S., Vazey, E. M., Kells, A. P., McGregor, A. L., Hughes, S. M., & Connor, B. (2007). Temporal profile of subventricular zone progenitor cell migration following quinolinic acid–induced striatal cell loss. Neuroscience, 146(4), 1704-1718. doi: 10.1016/j.neuroscience.2007.03.011

Chen, K., Henry, R. A., Hughes, S. M., & Connor, B. (2007). Creating a neurogenic environment: The role of BDNF and FGF2. Molecular & Cellular Neuroscience, 36(1), 108-120. doi: 10.1016/j.mcn.2007.06.004

Henry, R. A., Hughes, S. M., & Connor, B. (2007). AAV-mediated delivery of BDNF augments neurogenesis in the normal and quinolinic acid-lesioned adult rat brain. European Journal of Neuroscience, 25(12), 3513-3525. doi: 10.1111/j.1460-9568.2007.05625.x

Vazey, E. M., Chen, K., Hughes, S. M., & Connor, B. (2006). Transplanted adult neural progenitor cells survive, differentiate and reduce motor function impairment in a rodent model of Huntington's disease. Experimental Neurology, 199(2), 384-396. doi: 10.1016/j.expneurol.2006.01.034

Hughes, S. M., Moroni-Rawson, P., Jolly, R. D., & Jordan, T. W. (2001). Sub-mitochondrial distribution and delayed proteolysis of subunit c of the H+-transporting ATP synthase in ovine ceroid-lipofuscinosis. Electrophoresis, 22, 1785-1794.

^ Top of page

Conference Contribution - Published proceedings: Abstract

Mathiesen, S. N., Ohline, S. M., Wicky, H. E., Cheong, M. Y. I., Abraham, W. C., & Hughes, S. M. (2019). Assessing the efficacy of a novel adeno-associated viral capsid in targeting the brain. New Zealand Medical Journal, 132(1499), (pp. 87). Retrieved from https://www.nzma.org.nz/journal

Mathiesen, S. N., Schoderboeck, L., Ohline, S. M., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2018). Assessing the efficacy of adeno-associated viral vectors in targeting the brain. In K. Hillman (Ed.), Proceedings of the 36th International Australasian Winter Conference on Brain Research. (pp. 63). AWCBR. Retrieved from https://www.otago.ac.nz/awcbr

Parr-Brownlie, L. C., Sizemore, R. J., Smither, R. A., Hughes, S. M., & Bosch-Bouju, C. (2018). Substania nigra pars reticulata-ventroanterior motor thalamus synpases are altered in parkinsonian rats. Proceedings of the Society for Neuroscience 48th Annual Meeting. 146.13/II17. SfN. Retrieved from https://www.sfn.org

Oorschot, D. E., Seo, S., Bilkey, D. K., Hughes, S. M., Parr-Brownlie, L. C., & Sizemore, R. J. (2018). Synaptic triad arrangement within an aversive pathway in the normal uninjured brain. In K. Hillman (Ed.), Proceedings of the 36th International Australasian Winter Conference on Brain Research. (pp. 29). AWCBR. Retrieved from https://www.otago.ac.nz/awcbr

Seo, S., Sizemore, R. J., Bilkey, D. K., Hughes, S. M., Parr-Brownlie, L. C., & Oorschot, D. E. (2018). Changes in midbrain dopamine circuitry in the maternal immune activation rat model of schizophrenia. In K. Hillman (Ed.), Proceedings of the 36th International Australasian Winter Conference on Brain Research. (pp. 30). AWCBR. Retrieved from https://www.otago.ac.nz/awcbr

Eboda, O. N., Ward, M. E., & Hughes, S. M. (2018). ATP13A2: Characterization of novel human iPS cell models of Parkinson's and Batten's disease. In K. Hillman (Ed.), Proceedings of the 36th International Australasian Winter Conference on Brain Research. (pp. 58). AWCBR. Retrieved from https://www.otago.ac.nz/awcbr

Parr-Brownlie, L. C., Smither, R. A., Sizemore, R. J., Wicky, H. E., Hughes, S. M., & Bosch-Bouju, C. (2018). Anatomical and physiological changes at basal ganglia-motor thalamus synapses in rat model of Parkinson's disease. In K. Hillman (Ed.), Proceedings of the 36th International Australasian Winter Conference on Brain Research. (pp. 67). AWCBR. Retrieved from https://www.otago.ac.nz/awcbr

Clare, A. J., Wicky, H. E., Dearden, P. K., Empson, R. M., & Hughes, S. M. (2017). Developmentally important transcription factor Fezf2 has a molecular and functional role in the mature brain. Proceedings of the Annual Conference of the Genetics Society of Australasia (GSA) with the New Zealand Society for Biochemistry and Molecular Biology (NZSBMB). 693. Retrieved from http://gen2017.w.events4you.currinda.com

Yip, J. J. R., Wicky, H. E., Best, H. L., Mace, P. D., & Hughes, S. M. (2017). Understanding the Batten disease associated protein CLN5. Proceedings of the Annual Conference of the Genetics Society of Australasia (GSA) with the New Zealand Society for Biochemistry and Molecular Biology (NZSBMB). 666. Retrieved from http://gen2017.w.events4you.currinda.com

Ganesh, M., Clare, A., Dragunow, M., Hughes, S., Clarkson, A., & Empson, R. (2017). Pericyte therapeutics to repair the stroke-lesioned brain. Proceedings of the University of Otago Student Research Symposium: Te Wānaka Rakahau: Ākoka. (pp. 28). Retrieved from http://www.otago.ac.nz/graduate-research/scholarships/otago643219.html

Clare, A. J., Day, R. C., Empson, R. M., & Hughes, S. M. (2017). Transcriptome profiling of layer 5 intratelencephalic-projection neurons in the mature mouse motor cortex. In B. Leitch (Ed.), Proceedings of the 35th International Australasian Winter Conference on Brain Research. (pp. 37). AWCBR. Retrieved from http://www.otago.ac.nz/awcbr/index.html

Best, H. L., White, K., Weimer, J. M., & Hughes, S. M. (2017). Therapeutic targeting of autophagy in neurodegenerative Batten disease. In B. Leitch (Ed.), Proceedings of the 35th International Australasian Winter Conference on Brain Research. (pp. 44). AWCBR. Retrieved from http://www.otago.ac.nz/awcbr/index.html

Wicky, H. E., Best, H. L., & Hughes, S. M. (2017). A role for secreted proteins in CLN6 Batten disease therapy. In B. Leitch (Ed.), Proceedings of the 35th International Australasian Winter Conference on Brain Research. (pp. 57). AWCBR. Retrieved from http://www.otago.ac.nz/awcbr/index.html

Ohline, S. M., Hegemann, R. U., Wake, K. L., Dinnunhan, M. F., Schweitzer, L., Hughes, S. M., & Abraham, W. C. (2017). Dormant adult-born neurons can be activated by exposure to an enriched environment. Proceedings of the Society for Neuroscience 47th Annual Meeting. 115.19/B27. SfN. Retrieved from https://www.sfn.org/

Yip, J. J. R., Wicky, H. E., Best, H. L., Mace, P. D., & Hughes, S. M. (2017). Understanding the Batten disease associated protein CLN5. In B. Leitch (Ed.), Proceedings of the 35th International Australasian Winter Conference on Brain Research. (pp. 51). AWCBR. Retrieved from http://www.otago.ac.nz/awcbr/index.html

Ganesh, M., Clare, A., Dragunow, M., Hughes, S., Clarkson, A., & Empson, R. (2017). Pericyte therapeutics to repair the stroke-lesioned brain. In B. Leitch (Ed.), Proceedings of the 35th International Australasian Winter Conference on Brain Research. (pp. 51). AWCBR. Retrieved from http://www.otago.ac.nz/awcbr/index.html

Abraham, W. C., Ohline, S. M., Hegemann, R. U., Wake, K. L., Dinnunhan, M. F., Schweitzer, L., & Hughes, S. M. (2016). Do adult-born granule cells "retire"? Proceedings of the Society for Neuroscience 46th Annual Meeting. 740.27/III30. SfN. Retrieved from https://www.sfn.org/

Clare, A. J., Day, R. C., Empson, R. M., & Hughes, S. M. (2016). Transcriptome profiling of Fezf2 expressing intratelencephalic projection neurons in the mature mouse motor cortex. Proceedings of the 16th International Conference of Biochemistry and Molecular Biology (IUBMB): Signalling Pathways in Development, Disease and Aging. (pp. 78). Retrieved from http://www.iubmb2016.org

Ohline, S. M., Hegemann, R. U., Wake, K. L., Dinnunhan, M. F., Wilson, A. D., Schoderböck, L., Hughes, S. M., & Abraham, W. C. (2016). Adult born neurons: Do cells “retire” and does developmental age matter? New Zealand Medical Journal, 129(1442), (pp. 97). Retrieved from https://www.nzma.org.nz/journal

More publications...