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Associate Professor Stephanie Hughes

Stephanie HughesResearch in Associate Professor Hughes’ lab is aimed at identifying molecular pathways that regulate neuronal development, maintenance and disease. Her current research focuses on transcription factors of the forebrain embryonic zinc finger family and their role in neuronal development and maintenance. She is also investigating the molecular consequences of mutations in Batten disease, a childhood neurodegenerative disorder. The lab is developing tools and gene therapy vectors for Batten, Alzheimer’s and Parkinson’s disease.

Funding is provided by the Marsden Fund, the Health Research Council of New Zealand, the Neurological Foundation of New Zealand, the Otago Medical Research Foundation and the Otago School of Medical Sciences Deans Bequest Fund.

Find out more about Associate Professor Stephanie Hughes’ research

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Publications

Basak, I., Wicky, H. E., McDonald, K. O., Xu, J. B., Palmer, J. E., Best, H. L., … Schoderboeck, L., & Hughes, S. M. (2021). A lysosomal enigma CLN5 and its significance in understanding neuronal ceroid lipofuscinosis. Cellular & Molecular Life Sciences. Advance online publication. doi: 10.1007/s00018-021-03813-x

Klionsky, D. J., Abdel-Aziz, A. K., Abdelfatah, S., Abdellatif, M., Abdoli, A., Abel, S., … Hughes, S. M., … Katare, R. G., … Tong, C.-K. (2021). Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition). Autophagy. Advance online publication. doi: 10.1080/15548627.2020.1797280

Ryan, M., Tan, V. T. Y., Thompson, N., Guévremont, D., Mockett, B. G., Tate, W. P., Abraham, W. C., Hughes, S. M., & Williams, J. (2021). Lentivirus-mediated expression of human secreted amyloid precursor protein-alpha promotes long-term induction of neuroprotective genes and pathways in a mouse model of Alzheimer’s disease. Journal of Alzheimer's Disease, 79, 1075-1090. doi: 10.3233/JAD-200757

Mathiesen, S. N., Schoderboeck, L., Ohline, S. M., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2020). Improved gene transfer for the treatment of neurological disease using modified viral vector AAV-PHP.eB. New Zealand Medical Journal, 133(1527), (pp. 126-127). Retrieved from https://www.nzma.org.nz/journal

Best, H. L., Clare, A. J., McDonald, K. O., Wicky, H. E., & Hughes, S. M. (2020). An altered secretome is an early marker of the pathogenesis of CLN6 Batten disease. Journal of Neurochemistry. Advance online publication. doi: 10.1111/jnc.15285

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

Chapter in Book - Research

Klionsky, D. J., Abdel-Aziz, A. K., Abdelfatah, S., Abdellatif, M., Abdoli, A., Abel, S., … Hughes, S. M., … Katare, R. G., … Tong, C.-K. (2021). Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition). Autophagy. Advance online publication. doi: 10.1080/15548627.2020.1797280

Journal - Research Article

Ryan, M., Tan, V. T. Y., Thompson, N., Guévremont, D., Mockett, B. G., Tate, W. P., Abraham, W. C., Hughes, S. M., & Williams, J. (2021). Lentivirus-mediated expression of human secreted amyloid precursor protein-alpha promotes long-term induction of neuroprotective genes and pathways in a mouse model of Alzheimer’s disease. Journal of Alzheimer's Disease, 79, 1075-1090. doi: 10.3233/JAD-200757

Journal - Research Article

Best, H. L., Clare, A. J., McDonald, K. O., Wicky, H. E., & Hughes, S. M. (2020). An altered secretome is an early marker of the pathogenesis of CLN6 Batten disease. Journal of Neurochemistry. Advance online publication. doi: 10.1111/jnc.15285

Journal - Research Article

Huber, R. J., Hughes, S. M., Liu, W., Morgan, A., Tuxworth, R. I., & Russell, C. (2020). The contribution of multicellular model organisms to neuronal ceroid lipofuscinosis research. Biochimica et Biophysica Acta: Molecular Basis of Disease, 1866, 165614. doi: 10.1016/j.bbadis.2019.165614

Journal - Research Article

Mathiesen, S. N., Lock, J. L., Schoderboeck, L., Abraham, W. C., & Hughes, S. M. (2020). CNS transduction benefits of AAV-PHP.eB over AAV9 are dependent on administration route and mouse strain. Molecular Therapy: Methods & Clinical Development, 19, 447-458. doi: 10.1016/j.omtm.2020.10.011

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, 27(10), 1836-1847. doi: 10.1016/j.ymthe.2019.06.015

Journal - Research Article

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

Journal - Research Article

Morrissey, J. A., 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, 157, 107670. doi: 10.1016/j.neuropharm.2019.107670

Journal - Research Article

Schoderboeck, L., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2019). Genetic targeting and chemogenetic inhibition of newborn neurons. Human Gene Therapy Methods, 29(6), 259-268. doi: 10.1089/hgtb.2018.182

Journal - Research Article

Clare, A. J., Day, R. C., Empson, R. M., & Hughes, S. M. (2018). Transcriptome profiling of layer 5 intratelencephalic projection neurons from the mature mouse motor cortex. Frontiers in Molecular Neuroscience, 11, 410. doi: 10.3389/fnmol.2018.00410

Journal - Research Article

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, 26(10), 2366-2378. doi: 10.1016/j.ymthe.2018.07.015

Journal - Research Article

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, 223(7), 3213-3228. doi: 10.1007/s00429-018-1685-2

Journal - Research Article

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

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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.

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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

Journal - Research Article

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.

Journal - Research Article

Basak, I., Wicky, H. E., McDonald, K. O., Xu, J. B., Palmer, J. E., Best, H. L., … Schoderboeck, L., & Hughes, S. M. (2021). A lysosomal enigma CLN5 and its significance in understanding neuronal ceroid lipofuscinosis. Cellular & Molecular Life Sciences. Advance online publication. doi: 10.1007/s00018-021-03813-x

Journal - Research Other

Mathiesen, S. N., Schoderboeck, L., Ohline, S. M., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2020). Improved gene transfer for the treatment of neurological disease using modified viral vector AAV-PHP.eB. New Zealand Medical Journal, 133(1527), (pp. 126-127). Retrieved from https://www.nzma.org.nz/journal

Conference Contribution - Published proceedings: Abstract

Barnett, S. C., Young, C. K., Perry, B. A. L., McNaughton, N., Hughes, S. M., Parr-Brownlie, L. C., & Dalrymple-Alford, J. C. (2019). Optogenetic stimulation of the anterior thalamic nuclei ameliorates impaired spatial memory in rats with mammillothalamic tract lesions. In K. Hillman (Ed.), Proceedings of the 37th International Australasian Winter Conference on Brain Research (AWCBR). (pp. 27). Retrieved from http://www.otago.ac.nz/awcbr

Conference Contribution - Published proceedings: Abstract

Basak, I., Hansen, R., Eboda, O., Ward, M. E., & Hughes, S. M. (2019). Characterisation of CLN5 Batten disease in induced pluripotent stem cell derived human neurons. Proceedings of the 39th Australasian Neuroscience Society (ANS) Annual Scientific Meeting. 12146. Retrieved from https://www.ans.org.au

Conference Contribution - Published proceedings: Abstract

Chawdhary, B., Kim, S. H., Hughes, S., Parr-Brownlie, L., Waldvogel, H. J., Faull, R. L. M., & Kwakowsky, A. (2019). Miniaturized wireless optolectronic subdermal implants: A novel device for the optogenetic stimulation of hippocampal neurons. In K. Hillman (Ed.), Proceedings of the 37th International Australasian Winter Conference on Brain Research (AWCBR). (pp. 53). Retrieved from http://www.otago.ac.nz/awcbr

Conference Contribution - Published proceedings: Abstract

Eboda, O. N., Basak, I., Ward, M. E., & Hughes, S. M. (2019). ATP13A2: Characterization of a novel human iPS cell model of Parkinson's and Batten disease. Proceedings of the 39th Australasian Neuroscience Society (ANS) Annual Scientific Meeting. 13203. Retrieved from https://www.ans.org.au

Conference Contribution - Published proceedings: Abstract

Ganesh, M., Clare, A. J., Dragunow, M., Hughes, S. M., Clarkson, A. N., & Empson, R. M. (2019). Deciphering the fate of integrate pericytes in mouse motor cortex. In K. Hillman (Ed.), Proceedings of the 37th International Australasian Winter Conference on Brain Research (AWCBR). (pp. 35). Retrieved from http://www.otago.ac.nz/awcbr

Conference Contribution - Published proceedings: Abstract

Ganesh, M., Clare, A. J., Dragunow, M., Hughes, S. M., Clarkson, A. N., & Empson, R. M. (2019). Deciphering the fate of integrated pericytes in mouse motor cortex. Proceedings of the 39th Australasian Neuroscience Society (ANS) Annual Scientific Meeting. 12491. Retrieved from https://www.ans.org.au

Conference Contribution - Published proceedings: Abstract

Kleffmann, T., Tate, W., & Hughes, S. (2019). Does secreted amyloid precursor protein alpha affect the global proteome of astrocytes? Proceedings of the 39th Australasian Neuroscience Society (ANS) Annual Scientific Meeting. 13247. Retrieved from https://www.ans.org.au

Conference Contribution - Published proceedings: Abstract

Kwakowsky, A., Chawdhary, B., Kim, S., Hughes, S., Parr-Brownlie, L., Waldvogel, H., & Faull, R. (2019). Miniaturized wireless optoelectronic subdermal implants: A novel device for the optogenetic stimulation of hippocampal neurons. Proceedings of the 39th Australasian Neuroscience Society (ANS) Annual Scientific Meeting. 12508. Retrieved from https://www.ans.org.au

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

Conference Contribution - Published proceedings: Abstract

Mathiesen, S. N., Schoderboeck, L., Ohline, S. M., Wicky, H. E., Abraham, W. C., & Hughes, S. M. (2019). Investigating enhanced gene transfer to the mouse central nervous system using modified viral vectors. Proceedings of the Society for Neuroscience Annual Meeting. 175.13/BB83. SfN. Retrieved from https://www.sfn.org

Conference Contribution - Published proceedings: Abstract

Ohline, S. M., Chan, C. H., Whelan, J., Smith, H., Logan, B. J., Morrissey, J. A., Schoderboeck, L., Peppercorn, K., Tate, W. P., Hughes, S. M., & Abraham, W. C. (2019). Stimulation of neurogenesis by a potential therapeutic protein. In K. Hillman (Ed.), Proceedings of the 37th International Australasian Winter Conference on Brain Research (AWCBR). (pp. 57). Retrieved from http://www.otago.ac.nz/awcbr

Conference Contribution - Published proceedings: Abstract

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