Stephanie Hughes

Stephanie Hughes
Academic background
Alma materVictoria University of Wellington
Thesis
  • Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses (1999)
Doctoral advisorThomas William (Bill) Jordan
Academic work
InstitutionsUniversity of Otago, University of Otago, University of Auckland, Victoria University of Wellington, University of Iowa

Stephanie Margaret Hughes is a New Zealand molecular neurobiologist, and is a full professor at the University of Otago, specialising in gene therapy for the fatal childhood disorders known as Batten disease.

Academic career

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Hughes grew up in Wellington, and is from a family with a four-generation history of land surveying. Hughes attended Victoria University of Wellington, where she first intended to study maths and statistics, but took an honours degree in genetics and molecular biology instead.[1] Hughes completed a PhD titled Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses at Victoria University of Wellington, supervised by Bill Jordan.[2][1] Jordan introduced Hughes to Batten disease, which is a group of fatal inherited childhood neurological degenerative disorders. Hughes began studying Batten disease in the livers of a sheep model developed by Bob Jolly.[1] During her studies, Hughes realised she would need to grow sheep brain cells in laboratory culture, and travelled to the UK for training in the technique.[1] Hughes then began looking at gene therapy during her postdoctoral work at the University of Iowa, learning how to make viral vectors and conduct gene therapy in a mouse model.[1] Hughes worked at the University of Auckland for several years before joining Otago in 2008 as a research group leader.[1] She was promoted to associate professor in 2019 and full professor in 2023.[3][1][4] As of 2024 Hughes is the Director of the Brain Health Research Centre at Otago.[4][5]

Hughes's research focuses on the neurobiology of Batten disease. Hughes and her research group developed a gene therapy for one form of the disease, CLN5, which has progressed to clinical trials in humans.[4][1][6][7][8] Hughes's laboratory also developed human induced pluripotent stem cells which are better models than animal cells, and can be used to study other disorders, such as Alzheimer's disease.[1]

Hughes has received research funding from the Marsden fund, the Health Research Council, the Neurological Foundation and Cure Kids.[4]

Selected works

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Scholia has a profile for Stephanie Hughes (Q61152085).

References

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  1. ^ a b c d e f g h i Biochemistry, Department of (28 April 2023). "Treatment for a rare genetic disease leads to professorial promotion". www.otago.ac.nz. Retrieved 12 April 2024.
  2. ^ Hughes, Stephanie Margaret (1999). Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses (PhD thesis). Victoria University of Wellington.
  3. ^ Tourism, Department of (12 December 2018). "University of Otago announces academic promotions". www.otago.ac.nz. Retrieved 12 April 2024.
  4. ^ a b c d University of Otago, Wellington (16 December 2022). "Otago announces 39 new professors". www.otago.ac.nz. Retrieved 12 April 2024.
  5. ^ Otago, University of (3 April 2023). "Inaugural Professorial Lecture – Professor Stephanie Hughes". www.otago.ac.nz. Retrieved 12 April 2024.
  6. ^ Scott, Matthew (31 July 2022). "Kiwi researchers leap forward in hunt for fatal disease cure". Newsroom. Retrieved 12 April 2024.
  7. ^ Gilchrist, Shane (14 October 2015). "A glimmer of hope". Otago Daily Times Online News. Retrieved 12 April 2024.
  8. ^ Wenley, Sally (21 November 2014). "Woolly thinking a real life-saver". www.stuff.co.nz. Retrieved 12 April 2024.
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