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Protein linked to Huntington's disease brain cell death

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Scientists have linked a small protein to the deterioration of brain cells in patients suffering from Huntington’s disease.

Huntington’s is an inherited neurodegenerative disorder which can start with a lack of co-ordination and decline into abnormal movements, reduction in mental abilities, psychiatric problems and dementia.

It has long been known that another faulty protein, called huntingtin, is the primary cause of the disease.

But experts have struggled to explain why huntingtin only kills cells in the part of the brain that controls movement even though it is found to accumulate in cells throughout the body.

Researchers at Johns Hopkins University, in the US, working on mice found the tiny Rhes protein attaches itself more strongly to the huntingtin than proteins in other cells. This led to the death of the affected brain cells within 48 hours.

Writing in Science magazine, Professor Solomon Snyder said: ‘It’s always been a mystery why, if the protein made by the HD gene is seen in all cells of the body, only the brain, and only a particular part of the brain, the corpus striatum, deteriorates.

‘By finding the basic culprit, the potential is there to develop drugs that target it and either prevent symptoms or slow them down.’

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