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Study offers clues to beating hearing loss

Researchers at the University of Leeds have made a significant step forward in understanding the causes of some forms of deafness.

The Leeds team has discovered that the myosin 7 motor protein - found in the tiny hairs of the inner ear that pick up sound - moves and works in a different way from many other myosins. 

Dr Michelle Peckham from the University of Leeds' Faculty of Biological Sciences says: "We're really excited by this discovery as it could lead to new insights into certain forms of deafness. Mutations in this protein have been linked to hearing loss, particularly of the type connected to Usher syndrome, which is a form of degenerative deaf-blindness."

There are around 40 myosin motor proteins in the human body, the most familiar of which is the type of myosin found in skeletal and heart muscle.  But all cells have many different kinds of myosin.

Dr Peckham says: "What's exciting about our findings is that we have found that, unlike muscle myosins, which have two heads, myosin 7 only has one.  What's more, for the first time we've found how this myosin can be switched on and switched off.  When switched off, the tip of its tail curls round and contacts the head, and switched on this contact is broken and the myosin stretches out. This knowledge should help inform any further studies into how a mutation can create problems in hearing."

Follow-up studies could include a more detailed analysis of the role of myosin 7 in Usher syndrome, an inherited genetic condition, which affects hearing, sight and balance. It can vary in its severity; in some cases a child may be born deaf and their sight may deteriorate during childhood, whilst in others the syndrome can go undetected into the teens when hearing and sight usually begins to deteriorate.

There is no cure for Usher syndrome and sufferers are usually offered assistance in managing their disabilities.

Dr Peckham says: "Our studies on how normal myosin 7 works pave the way for understanding how a defective myosin 7 protein in Usher patients results in deafness."

Dr Peckham worked with colleagues Professor Peter Knight and Dr Tom Baboolal from the Faculty of Biological Sciences. This is a collaborative study with Prof Jim Seller's group at The National Heart, Lung and Blood Institute in the USA and has been funded by the BBSRC. It is published in the Proceedings of the National Academy of Sciences Online Early Edition this week.

For further information:

Please contact the University of Leeds Press Office on +44 (0)113 343 4031 or email pressoffice@leeds.ac.uk

Note to editors:

1. The paper, A FERM domain autoregulates Drosophilia myosin 7a activity is published online and is available from Campuspr or by emailing pnasnews@nas.edu

2. The Faculty of Biological Sciences at the University of Leeds is one of the largest in the UK, with over 150 academic staff and over 400 postdoctoral fellows and postgraduate students.

The Faculty is ranked 4th in the UK (Nature Journal, 457 (2009) doi:10.1038/457013a) based on results of the 2008 Research Assessment Exercise (RAE).  The RAE feedback noted that "virtually all outputs were assessed as being recognized internationally, with many (60%) being internationally excellent or world-leading" in quality.  The Faculty's research grant portfolio totals some £60M and funders include charities, research councils, the European Union and industry.  http://www.fbs.leeds.ac.uk/

3. The University of Leeds is one of the largest higher education institutions in the UK with more than 30,000 students from 130 countries and a turnover of £450m. The University is a member of the Russell Group of research-intensive universities and the 2008 Research Assessment Exercise showed it to be among the top UK research powerhouses. The University's vision is to secure a place among the world's top 50 by 2015. http://www.leeds.ac.uk/

4. The Biotechnology and Biological Sciences Research Council (BBSRC) is the UK funding agency for research in the life sciences. Sponsored by Government, BBSRC annually invests around £420 million in a wide range of research that makes a significant contribution to the quality of life for UK citizens and supports a number of important industrial stakeholders including the agriculture, food, chemical, healthcare and pharmaceutical sectors. BBSRC carries out its mission by funding internationally competitive research, providing training in the biosciences, fostering opportunities for knowledge transfer and innovation and promoting interaction with the public and other stakeholders on issues of scientific interest in universities, centres and institutes.

The Babraham Institute, Institute for Animal Health, Institute of Food Research, John Innes Centre and Rothamsted Research are Institutes of BBSRC. The Institutes conduct long-term, mission-oriented research using specialist facilities. They have strong interactions with industry, Government departments and other end-users of their research.  For more information see: http://www.bbsrc.ac.uk/

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