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Department of Biochemistry profile

Dr Stephanie Hughes

PositionSenior Lecturer
DepartmentDepartment of Biochemistry
QualificationsBSc(Hons) PhD
Research summaryNeural Development and Disease

Research

Research in our lab is aimed at identifying molecular pathways that regulate neuronal development, maintenance and disease. Our 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.

Scientific image. Scientific image. Scientific image.

Positions available

Enquires about projects from prospective graduate students and postdoctoral fellows are welcome.

Information about scholarships for New Zealand postgraduate students go to the University of Otago's website - Postgraduate and Scholarship Awards. University of Otago's website for information about funding for International Students.

Funding

  • Marsden Fund
  • HRC
  • Otago Medical Research Foundation
  • Otago School of Medical Sciences Dean's Bequest Fund

Publications

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

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

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

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

Schoderboeck, L., Riad, S., Bokor, A. A., 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

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

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Journal - Research Article

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

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

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

Schoderboeck, L., Riad, S., Bokor, A. A., 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

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

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

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

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

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

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

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

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.

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.

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