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Scientists gain insight into motor neurone disease

19 November 2008

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bbc.co.uk/1/hi/health/7733202.stm" target="_self">BBC news - Motor neurone disease clue found
  • BBSRC
  • UCL and King's College London have identified a molecule that could be the key to understanding the cause of neurodegenerative diseases such as motor neurone disease (MND). The research was funded by the BBSRC (Biotechnology and Biological Sciences Research Council), the Medical Research Council and the Wellcome Trust.

    This insight opens up the possibilities for developing new treatments to treat these devastating progressive conditions. The research is published in the 'Proceedings of the National Academy of Sciences' (PNAS).

    Lead researcher Professor Patricia Salinas said: "For decades we have been studying how nerves communicate with their target muscles and we know that in diseases like MND the sites of contact between nerves and muscles become weak. However, many mysteries remain as to how these contacts form under normal circumstances and therefore it has been very difficult to see what has gone wrong in MND. The work we are publishing today puts another important piece of the puzzle in place and offers up a new possibility for developing drugs to treat MND and other neurodegenerative diseases."

    Professor Salinas, with her husband Dr Simon Hughes (of the MRC) has found that a signalling molecule called Wnt3 plays a crucial role in creating the connections, or synapses, between nerves and the muscles they control. It does this by assisting another molecule called Agrin, which coordinates construction of the synapse and organises the elements that make up the connection.

    Professor Salinas continued: "Without properly formed synapses, the muscle cannot receive the nerve signal that tells it to contract and hence we see the muscle weakness that is classic in MND. If we can build up a thorough picture to show how synapses are normally formed between nerves and muscles, we can start to look for any elements that aren't working properly in people with MND. This might also lead to strategies for nerve repair after an injury."

    The team of researchers have looked at function of Wnt signals in chickens, mice and in cells and in all three cases it was shown to enhance the effectiveness of Agrin.

    Professor Salinas added: "Chickens that don't have the Wnt signal in their developing wings have all of the muscle tissue that we would expect to see, but they don't make strong connections between nerves and muscles. So we know that Wnt is definitely affecting synapse formation rather than anything else to do with muscles. Now that we understand the role Wnt plays, we can begin to explore any role it plays in MND and whether it could be a good target for treating this type of neurodegenerative disease."

    Read more about this story on the BBC website using the link above.