Stephanie Hughes | |
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Academic background | |
Alma mater | Victoria University of Wellington |
Thesis |
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Doctoral advisor | Thomas William (Bill) Jordan |
Academic work | |
Institutions | University 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.
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]