Scientists have discovered a rogue gene in familial motor neuron disease
Families suffering from a history of motor neuron disease have helped an international scientific team locate a new gene linked to the incurable disease. The investigators studied a large group of Finnish patients and a family from Gwent, South Wales who have lost many relatives to early onset motor neuron disease (MND) and the neurodegenerative disease frontotemporal dementia, also known as Pick's disease. The team, including scientists from The University of Manchester, Cardiff University and UCL (University College London), discovered that both the Gwent family and the Finnish patients share a changed genetic segment on the short arm of chromosome 9. Five thousand people in the UK have MND, and well-known sufferers have included David Niven and Don Revie. The disease is progressive and fatal, with an average survival from onset of symptoms of between two and five years. Scientists have been studying chromosome 9 for some years but until now have not been able to pinpoint the gene that causes motor neuron disease and frontotemporal dementia. The new discovery identifies the critical gene change as an expanded sequence of DNA repeats; it is currently unclear why some people with the mutation get frontotemporal dementia while others get MND.
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