Copenhagen, Denmark 9th May, 2012: Grete Lundbeck European Brain Research Foundation announced today that The Brain Prize is presented by Her Majesty the Queen of Denmark to Christine Petit and Karen Steel:
'for their unique, world-leading contributions to our understanding of the genetic regulation of the development and functioning of the ear, and for elucidating the causes of many of the hundreds of inherited forms of deafness'.
Prestigious international prize
The award ceremony takes place at the Royal Library, Black Diamond, in Copenhagen in front of a specially invited audience representing Danish as well as international neuroscience, last year's prize winners and Mr Morten Østergaard, Minister for Science, Innovation and Higher Education. This year's award is only the second award of this Prize. Yet it has already been recognized as belonging to the most prestigious international research prizes and it is the largest international prize for neuroscience.
Dr Nils Axelsen, Chairman of the Board of Directors of the Foundation said: It is our ambition that this Prize will be not only an international beacon for brain research but also a cherished icon for the Danish people. Therefore, we are extremely pleased that Her Majesty the Queen has shown the Foundation, the prize-winners and the Danish people the great honour to present the Prize this year. I am sure that this will also help further advance the recognition and the prestige of the prize at a national as well as an international level.
Fundamental research needed
About the work recognised by The Brain Prize 2012 Professor Colin Blakemore of Oxford University, Chairman of the Selection Committee, said: 'Karen Steel and Christine Petit are at the forefront of efforts to understand the molecular mechanisms of the specialised hair cells in the inner ear, whose extraordinary sensitivity to mechanical stimulation underpins the senses of hearing and balance. Together, the work of these two Europeans scientists illustrates the value and power of interdisciplinary approaches in neuroscience, and the way in which cutting-edge fundamental research is needed to understand complex clinical problems and to accelerate benefit for patients'
Video available
Facts:
Inherited conditions render one in a thousand children deaf at birth, and cause as many again to become deaf before maturity, leading to delay or failure in the acquisition of speech, and frequently to disadvantages in communication and learning. Genetic anomalies also contribute to many age-related and progressive forms of hearing loss. About one-tenth of the population in the developed world suffers from significant hearing impairment, which has an enormous impact on individuals and on society.
Bios:
Christine Petit, Professor at Collège de France, holding the Chair of Genetics and Cellular Physiology, head of the Genetics and Physiology of Hearing laboratory at the Institut Pasteur in Paris, also affiliated to INSERM, is a geneticist and a neurobiologist. She conceived and pioneered a genetic strategy to assess the molecular mechanisms underlying the differentiation and physiology of the human cochlea. She overcame the difficulties specific to linkage analysis for the study of deafness and mapped to human chromosomes the first two genes responsible for hereditary congenital deafness. She has identified the causative genes for about 20 inherited forms of deafness, initially by an innovative candidate gene approach. Together with her colleagues, she also unravelled the roles of most of the proteins encoded by these genes, namely in the sensory hair cells, their stimulating gel, and the supporting cells, by multidisciplinary studies of engineered mouse models. Her work shed light on the functions mediated through various fibrous links within the hair bundle, the mechanoreceptive structure that detects sound. Her work on Usher syndrome (deafness and blindness), demonstrated that all Usher 1 proteins as well as all Usher 2 proteins form protein networks enabling the links they form to shape the hair bundle. She also identified some Usher 1 proteins as components of the mechanotransduction machinery. Christine Petit's discoveries have already had a significant impact on medical practice, with respect to diagnosis, genetic counselling and therapeutic decisions, by indicating which patients are likely to benefit from cochlear implants or hearing aids.
Karen Steel, Professor and Principal Investigator for the Genetics of Deafness, and founder of the Mouse Genetics Programme at the Wellcome Trust Sanger Institute, Hinxton, near Cambridge, UK, has pioneered the use of mouse models, starting with her painstaking description of the characteristics of mutant mice with disorders of balance and hearing. Her early work on cochlear function and hair cell degeneration in the mutant deafness mouse led to identification of the gene called Tmc1, the human homologue of which is mutated in several forms of hereditary deafness. She and her colleagues went on to identify causative genes in nearly 30 forms of hearing disorder in mice, most of which have been linked to human conditions. In particular, they described the genes for myosin-7a and cadherin-23, which are key components in the transduction mechanism in hair cells, and mutations of which underlie forms of Usher Syndrome. Karen Steel is internationally recognized for her generous and altruistic approach to science. Working with a consortium of European researchers, she has established, catalogued and made freely available to other researchers several hundred mouse mutant lines, which have facilitated research in several areas of neuroscience around the world.
About the Brain Prize
The Brain Prize - € 1 million is awarded by Grete Lundbeck European Brain Research Foundation, a charitable, non-profit organization.
The Brain Prize is a personal prize awarded to one or more scientists who have distinguished themselves by an outstanding contribution to European neuroscience.
For more information please contact:
Kim Krogsgaard, MD, DMSc
Director
Mobile: +45 2014 8384
Phone: +45 3917 8240
kk@thebrainprize.org
or
Nils Axelsen, MD, DMsc
Chairman
Mobile: +45 40548646
Grete Lundbeck European Brain Research Foundation
Ole Maaløes Vej 3
2200 Copenhagen N
Denmark
www.thebrainprize.org