Karen Steel

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Karen Penelope Steel FRS FMedSci [1] is a British scientist who studies the genetics of deafness, using the mouse as a model to identify the genes involved and to understand the molecular, cellular and physiological mechanisms involved. She is Professor of Sensory Function at the Wolfson Centre for Age-Related Diseases, King's College London. [2] Previously she was Principal Investigator of the Genetics of Deafness research programme at the Wellcome Trust Sanger Institute. [3]

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She had a leading role in the collaboration that uncovered Myo7a, the first gene to be implicated in deafness in mice and in humans. Most recently, she led the discovery of Mir-96 microRNA that is implicated in progressive hearing loss in mice and humans. [3]

Together with Professor Christine Petit, Steel won the Royal Society Brain Prize 2012, for pioneering work on the genetics of hearing and deafness. [3]

Early life

In her early academic years, Steel was originally interested in studying English. Her first exposure to biology, and also what solidified her biological science career path, was in sixth grade. Steel had the opportunity to hear Dorothy Hodgkin speak at Bristol University about insulin, marking her first time encountering a woman in science. This experience greatly influenced her interest in studying genetics. [4]

Education

Steel obtained her first degree from Leeds University. She then received her PhD from University College London for her investigatory work into the inner ear in several deaf mouse mutants. She set up the mouse genetics and deafness research programme at the newly established MRC Institute of Hearing Research in Nottingham. Following a second postdoc in Munich, she returned to Nottingham to lead mouse genetics research. [5]

Research

Steel studies the genetics behind deafness, mainly focusing on the genetics of mice in order to identify the genes involved as well as to further understand the molecular, cellular and physiological mechanisms involved with being deaf. [6] This led to Steel developing a screening technique, a triaging process, that allowed for the characterization of mutant mice that have hearing and imbalance issues. This led to ability to find and characterize the specific genes involved in these issues, with these issues being caused by altered hair cells or auditory parts. [4] Steel's mouse genetic project also involved knocking out various genes not extensively studied and observing whether these genes are essential or nonessential in the mouse hearing. Steel and her research group have published phenotypic descriptions of over 80 different mouse mutants. Currently, her research is focused on the progressive loss of auditory functions in which she uses the mouse models to diagram the timeline of auditory loss in humans. [5] She also looks for specific mutated genes that are known to contribute and lead to hearing loss, allowing her to target genes that could be potentially targeted in treatments for progressive hearing loss. As of 2014, Steel and her team has found genes involved in progressive hearing loss and has moved forward in identifying the various pathways that these genes are a part of. [7]

Awards and honours

The asteroid 24734 Kareness was named after Steel by its discoverer, Steel's brother.

Related Research Articles

Hearing loss Partial or total inability to hear

Hearing loss is a partial or total inability to hear. Hearing loss may be present at birth or acquired at any time afterwards. Hearing loss may occur in one or both ears. In children, hearing problems can affect the ability to acquire spoken language, and in adults it can create difficulties with social interaction and at work. Hearing loss can be temporary or permanent. Hearing loss related to age usually affects both ears and is due to cochlear hair cell loss. In some people, particularly older people, hearing loss can result in loneliness. Deaf people usually have little to no hearing.

Usher syndrome Recessive genetic disorder causing deafblindness

Usher syndrome, also known as Hallgren syndrome, Usher–Hallgren syndrome, retinitis pigmentosa–dysacusis syndrome or dystrophia retinae dysacusis syndrome, is a rare genetic disorder caused by a mutation in any one of at least 11 genes resulting in a combination of hearing loss and visual impairment. It is a major cause of deafblindness and is at present incurable.

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Myosin VIIA is protein that in humans is encoded by the MYO7A gene. Myosin VIIA is a member of the unconventional myosin superfamily of proteins. Myosins are actin binding molecular motors that use the enzymatic conversion of ATP - ADP + inorganic phosphate (Pi) to provide the energy for movement.

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USH1C

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Stephen D. M. Brown

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References

  1. ↑ "Professor Karen Steel FRS FMedSci". The Academy of Medical Sciences. Archived from the original on 2013-10-15. Retrieved 2013-10-19.
  2. ↑ "Karen Steel Biography". King's London: Karen Steel Biography. Retrieved 2015-10-13.
  3. 1 2 3 "Karen Steel elected Fellow of the Royal Society". Wellcome Trust Sanger Institute. 15 May 2009. Retrieved 2013-10-19.
  4. 1 2 Limited, The Company of Biologists (2011-11-01). "Mouse genetics for studying mechanisms of deafness and more: an interview with Karen Steel". Disease Models & Mechanisms. 4 (6): 716–718. doi: 10.1242/dmm.008813 . ISSN   1754-8403. PMC   3209639 . PMID   22065840.
  5. 1 2 3 "Biography - Karen Steel". Grethe Lundbeck’s European Brain Research Foundation . Retrieved 2014-01-31.
  6. ↑ "Professor Karen Steel". King's College of London. Retrieved 2014-01-31.
  7. ↑ "Prof Karen Steel - Genes and deafness". www.thenakedscientists.com. 2014-08-01. Retrieved 2019-11-21.
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