Stephanie Hughes
{{Short description|New Zealand neurobiologist}}
{{Use dmy dates|date=April 2024}}
{{Use New Zealand English|date=April 2024}}
{{Infobox scientist
| name = Stephanie Hughes
| native_name =
| native_name_lang =
| image = Stephanie Hughes 2025 (cropped).jpg
| alt =
| caption = Hughes in 2025
| birth_date = {{#statements:P569}}
| birth_place = {{#statements:P19}}
| death_date = {{#statements:P570}}
| death_place = {{#statements:P20}}
| other_names =
| field = Molecular neurobiology
| workplaces = University of Otago
| patrons =
| alma_mater = Victoria University of Wellington
| thesis1_title = Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses
| thesis1_url = https://tewaharoa.victoria.ac.nz/permalink/64VUW_INST/k63216/alma99180170744202386
| thesis1_year = 1999
| doctoral_advisor = {{#statements:P184}}
| academic_advisors = {{#statements:P1066}}
| doctoral_students =
| notable_students =
| known_for =
| influences =
| influenced =
| awards = {{#statements:P166}}
| signature =
| signature_alt =
| website =
| footnotes =
| spouse =
| children =
}}
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
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. Hughes completed a PhD titled Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses at Victoria University of Wellington, supervised by Bill Jordan.{{Cite thesis|title=Molecular pathology of subunit c storage in neuronal ceroid lipofuscinoses|last=Hughes|first=Stephanie Margaret|type=PhD thesis|publisher=Victoria University of Wellington|url=https://tewaharoa.victoria.ac.nz/permalink/64VUW_INST/k63216/alma993537494002386|date=1999}} 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. 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. 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. Hughes worked at the University of Auckland for several years before joining Otago in 2008 as a research group leader. She was promoted to associate professor in 2019 and full professor in 2023.{{Cite web |last=Tourism |first=Department of |date=12 December 2018 |title=University of Otago announces academic promotions |url=https://www.otago.ac.nz/news/newsroom/12-december-2018-university-of-otago-announces-academic-promotions |access-date=12 April 2024 |website=www.otago.ac.nz |language=en}}{{Cite web |last=Biochemistry |first=Department of |date=28 April 2023 |title=Treatment for a rare genetic disease leads to professorial promotion |url=https://www.otago.ac.nz/news/newsroom/treatment-for-a-rare-genetic-disease-leads-to-professorial-promotion |access-date=12 April 2024 |website=www.otago.ac.nz |language=en}}{{Cite web |last=University of Otago |first=Wellington |date=16 December 2022 |title=Otago announces 39 new professors |url=https://www.otago.ac.nz/news/newsroom/otago-announces-39-new-professors |access-date=12 April 2024 |website=www.otago.ac.nz |language=en}} As of 2024 Hughes is the Director of the Brain Health Research Centre at Otago.{{Cite web |last=Otago |first=University of |date=3 April 2023 |title=Inaugural Professorial Lecture – Professor Stephanie Hughes |url=https://www.otago.ac.nz/news/events/inaugural-professorial-lecture-professor-stephanie-hughes |access-date=12 April 2024 |website=www.otago.ac.nz |language=en}}
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.{{Cite web |last=Scott |first=Matthew |date=31 July 2022 |title=Kiwi researchers leap forward in hunt for fatal disease cure |url=http://newsroom.co.nz/2022/07/31/kiwi-researchers-leap-forward-in-hunt-for-fatal-disease-cure/ |access-date=12 April 2024 |website=Newsroom |language=en-US}}{{Cite news |last=Gilchrist |first=Shane |date=14 October 2015 |title=A glimmer of hope |url=https://www.odt.co.nz/lifestyle/magazine/glimmer-hope |access-date=12 April 2024 |work=Otago Daily Times |language=en}}{{Cite web |last=Wenley |first=Sally |date=21 November 2014 |title=Woolly thinking a real life-saver |url=https://www.stuff.co.nz/science/63423984/woolly-thinking-a-real-life-saver |access-date=12 April 2024 |website=Stuff }} 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.
Hughes has received research funding from the Marsden fund, the Health Research Council, the Neurological Foundation and Cure Kids.
Selected works
{{scholia}}
- {{CiteQ|Q110176737}}
- {{CiteQ|Q39295382}}
- {{CiteQ|Q34028579}}
- {{CiteQ|Q74285552}}
- {{CiteQ|Q42678498|doi-access=free}}
- {{CiteQ|Q45300810}}
- {{CiteQ|Q45882544}}
References
{{reflist}}
External links
- [https://www.youtube.com/watch?v=MQ6Xs3x4QwQ Keeping it in the family: the search for a cure for childhood Batten disease]. Stephanie Hughes inaugural professorial lecture, 20 April 2023
{{Authority control}}
{{DEFAULTSORT:Hughes, Stephanie}}
Category:Year of birth missing (living people)
Category:New Zealand women academics
Category:Academic staff of the University of Otago
Category:Academic staff of the University of Auckland
Category:Victoria University of Wellington alumni
Category:New Zealand neuroscientists
Category:New Zealand women scientists