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Protein found to slow brain disease

United Press International


A protein that helps the growth of neurons and blood vessels appears to slow or even stop a genetic brain disease of the cerebellum, U.S. researchers say.

The disease, spinocerebellar ataxia type 1, strikes people in their 30s and 40s and causes degeneration of the cerebellum, the part of the brain controlling movement, medical researchers at Northwestern University said.

The disease is caused by a mutation in a protein called ataxin-1, which helps regulated a protein called vascular endothelial growth factor.

When Northwestern scientists replenished VEGF in the brains of a mouse model of the disease, the brains began to appear more normal, with an increase in connections between neurons, and the mice displayed improved balance, a university release said Monday.

"If you give VEGF early in the disease, you prevent degeneration later in life," Northwester Professor of Neurology Puneet Opal said. "We think VEGF increases the blood vessels in the brain but also directly prevents neurons from dying. These results hold the potential for future therapy."

Because patients are born with the mutation for the disease but don't show signs until midlife, the aging process appears to play a role in development of the disease, Opal said.

"There could be a connection between a patient's genetic mutation and their blood vessels not keeping up as they age," Opal said. "When we delivered VEGF to the brain and increased blood vessels, the disease stopped progressing in mice."

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