Dr Stephanie Hughes

Senior Lecturer
Steph 2
  • Biochemistry Department
  • School of Biomedical Sciences
  • University of Otago
  • P.O. Box 56
  • 710 Cumberland St
  • Dunedin 9054 , New Zealand
  • Tel.: 64 3 479-3761
  • Fax: 64 3 479-7866
  • Email:stephanie.hughes@otago.ac.nz

Neural Development and Disease Lab:

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.

 

sheep picture

zinc finger protein structure

fluorescent image of brain cells

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 & Scholarship Awards.
University of Otago's website for information about funding for International Students.

Funding:

Marsden Fund logo

 

Otago Medical Research Foundation
Otago School of Medical Sciences Deans Bequest Fund

PhD projects available in this laboratory

Awards

  • Nicole Neverman
  • 2014, OtagoSchool of Medical Sciences Postgraduate Symposium Speaker Award, Runner up
  • Sharleen Rae
  • 2011, University of Otago Health Science Research Forum Poster Prize, 1st place
  • This prize was awarded jointly to Sharleen Rae and Malinda Tantirigama from Physiology.
  • Katie Hope
  • 2010, Otago Medical programme Best Lab Demonstrator Award

Selected Publications

Hannah L Best, Nicole J Neverman, Hollie E Wicky, Nadia L Mitchell, Beulah Leitch, and Stephanie M Hughes., Characterisation of early changes in ovine CLN5 and CLN6 Batten disease neural cultures for the rapid screening of therapeutics., Neurobiology of Disease 2017 vol. 100 pp. 62-74., Link »

Rachel J Sizemore, Sonja Seeger-Armbruster, Stephanie M Hughes, and Louise C Parr-Brownlie., Viral vector-based tools advance knowledge of basal ganglia anatomy and physiology., J Neurophysiol 2016 vol. 115 (4) pp. 2124-2146., Link »

Malinda L S Tantirigama, Manfred J Oswald, Alison J Clare, Hollie E Wicky, Robert C Day, Stephanie M Hughes, and Ruth M Empson., Fezf2 Expression in Layer 5 Projection Neurons of Mature Mouse Motor Cortex., J Comp Neurol 2015, Link »

Louise C Parr-Brownlie, Clémentine Bosch-Bouju, Lucia Schoderboeck, Rachel J Sizemore, Wickliffe C Abraham, and Stephanie M Hughes., Lentiviral vectors as tools to understand central nervous system biology in mammalian model organisms., Front Mol Neurosci 2015 vol. 8 p. 14., Link »

Nicole J Neverman, Hannah L Best, Sandra L Hofmann, and Stephanie M Hughes., Experimental therapies in the neuronal ceroid lipofuscinoses., Biochimica et biophysica acta 2015, Link »

L Schoderboeck, S Riad, A M Bokor, H E Wicky, M Strauss, M Bostina, M J Oswald, R M Empson, and S.M. Hughes., Chimeric rabies SADB19-VSVg-pseudotyped lentiviral vectors mediate long-range retrograde transduction from the mouse spinal cord., Gene Therapy 2015, Link »

Sonja Seeger-Armbruster, Clémentine Bosch-Bouju, Shane T C Little, Roseanna A Smither, Stephanie M Hughes, Brian I Hyland, and Louise C Parr-Brownlie., Patterned, but not tonic, optogenetic stimulation in motor thalamus improves reaching in acute drug-induced parkinsonian rats., Journal of Neuroscience 2015 vol. 35 (3) pp. 1211-1216., Link »

Malinda L S Tantirigama, Manfred J Oswald, Celine Duynstee, Stephanie M Hughes, and Ruth M Empson, 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 2014 vol. 34 (12) pp. 4303-4308, Link »

Hughes, S. M., Hope, K. M., Xu, J. B., Mitchell, N. L., & Palmer, D. N., Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy., Neurobiology of Disease 2014 vol. 62 pp. 543-550, Link »

Stephanie M Hughes, Katie M Hope, Janet Boyu Xu, Nadia L Mitchell, and David N Palmer, Inhibition of storage pathology in prenatal CLN5-deficient sheep neural cultures by lentiviral gene therapy., Neurobiology of Disease Volume 62, Pages 543–550, Link »

Manfred J Oswald, Malinda L S Tantirigama, Ivo Sonntag, Stephanie M Hughes, and Ruth M Empson, Diversity of layer 5 projection neurons in the mouse motor cortex, Frontiers in Cellular Neuroscience 2013 vol. 7 p. , Link »

Reagan M Jarvis, Stephanie M Hughes, and Elizabeth C Ledgerwood, Peroxiredoxin 1 functions as a signal peroxidase to receive, transduce, and transmit peroxide signals in mammalian cells., Free Radical Biology And Medicine Volume 53, Issue 7, 1 October 2012, Pages 1522–1530, Link »

Linterman KS, Palmer DN, Kay GW, Barry LA, Mitchell N, McFarlane R, Black M, Sands MS, Hughes SM. Lentiviral-mediated gene transfer to the sheep brain – implications for gene therapy in Batten disease. Human Gene Therapy, in press, 2011. 

Dottori M, Tay C, Hughes SM. Neural development in human embryonic stem cells – applications of lentiviral vectors. Journal of Cellular Biochemistry, 112:1955-62, 2011.

Gibbons HM, Hughes SM, Van Roon-Mom W, Greenwood JM, Narayan PJ, Teoh HH, Bergin PM, Mee EW, Wood PC, Faull RLM, Dragunow M. Cellular composition of human glial cultures from adult biopsy brain tissue. Journal of Neuroscience Methods, 166:89-98, 2007

Chen KC, Henry RA, Hughes SM, Connor B. Creating a neurogenic environment: the role of BDNF and FGF2. Molecular and Cellular Neuroscience, 33:108-20, 2007

Chen KC, Hughes SM, Connor B. Neural Progenitor Cells Derived From The Adult Rat Subventricular Zone: Characterization And Transplantation. Cell Transplantation, In Press, 2007

Henry RA, Hughes SM, Connor B. AAV-Mediated Delivery of BDNF Augments Neurogenesis in the Normal and Quinolinic Acid Lesioned Adult Rat Brain. European Journal of Neuroscience, 25:3513-25, 2007

Gordon RJ, Tattersfield AS, Vazey EM, Kells AP, McGregor AL, Hughes SM, Connor B. Temporal Profile of Subventricular Zone Progenitor Cell Migration Following Quinolinic Acid Induced Striatal Cell Loss. Neuroscience 146:1704-1718, 2007

Kells AP, Henry RA, Hughes SM, Connor B. Verification of functional AAV-mediated neurotrophic and anti-apoptotic factor expression. Journal of Neuroscience Methods 161:291-300, 2007

Vazey EM, Chen KC, Hughes SM, Connor B. Transplanted Adult Neural Progenitor Cells Survive, Differentiate and Reduce Motor Function Impairment in a Rodent Model of Huntington's Disease. Experimental Neurology 199:384-396,2006

Alisky JM, Hughes SM, Davidson BL. Transduction of neurons lining the cerebral external capsule in mice with feline immunodeficiency virus based vectors. Neuroscience Letters 351:120-124, 2003.

Haskell R, Hughes SM*, Chiorini JA, Alisky JM, Davidson BL. Viral mediated delivery of the late-infantile neuronal ceroid lipofuscinosis gene, TTP-I to the mouse central nervous system. Gene Therapy 10:34-42, 2003. * co-first author

Brooks AI, Stein CS, Hughes SM, Heth JA, McCray PM, Jr, Sauter SL, Johnson JC, Cory-Slechta DA, Federoff HJ, Davidson BL. Functional correction of established central nervous system deficits in an animal model of lysosomal storage disease with feline immunodeficiency based vectors. Proceedings of the National Academy of Sciences, USA 99:6216-6221, 2002.

Hughes SM, Moussavi-Harami F, Sauter SL, Davidson BL. Viral-mediated gene transfer to mouse primary neural progenitor cells. Molecular Therapy 5:16-24, 2002.

Hughes SM, Moroni-Rawson P, Jolly RD, Jordan TW. Sub-mitochondrial distribution and delayed processing of subunit c of the H+-transporting ATP synthase in ovine ceroid lipofuscinosis. Electrophoresis 22:1785-1794, 2001.

Alisky JM, Hughes SM, Sauter SL, Jolly D, Dubensky TW Jr, Staber PD, Chiorini JA, Davidson BL. Transduction of murine cerebellar neurons with recombinant FIV and AAV5 vectors. NeuroReport 11:2669-2673, 2000.

Kay GW, Hughes SM, Palmer DN. In vitro culture of neurons from sheep with Batten disease. Molecular Genetics and Metabolism 67:83-88, 1999.

Hughes SM, Kay GW, Jordan TW, Rickards GK, Palmer DN. Disease-specific pathology in neurons from sheep affected with ceroid lipofuscinosis. Molecular Genetics and Metabolism 66:381-386, 1999.