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Neurone disease study

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Thursday, October 11, 2012
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Bath Chronicle

Scientists at the University of Bath are one step closer to understanding the role of one of the proteins that causes motor neurone disease.

The scientists studied a protein called angiogenin, which is present in the spinal cord and brain that protects neurones from cell death. Mutations in this protein have been found in sufferers of MND and are thought to play a key role in the progression of the condition.

MND triggers progressive weakness, muscle atrophy and muscle twitches and spasms, and affects around 5,000 people in the UK.

The team of cell biologists and structural biologists have, for the first time, produced images of the 3D structures of 11 mutant versions of angiogenin to see how the mutations changed the structure of the active part of the molecule, damaging its function.

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The study, published in the prestigious journal Nature Communications, provides insights into the causes of this disease and related conditions such as Parkinson's Disease.

The team also looked at the effects of the malfunctioning proteins on neurones grown from embryonic stem cells in the laboratory.

Dr Vasanta Subramanian, reader in biology and biochemistry, said: "We hope that the scientific community can use this new knowledge to help design new drugs that will bind selectively to the defective protein to protect the body from its damaging effects."

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