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Burke gets grant to study spinal muscular atrophy


Burke Medical Research Institute has received a federal grant to study a protein linked to spinal muscular atrophy, the leading genetic cause of infant mortality.

The National Institute of Neurological Disorders and Stroke awarded $475,000 to a team headed by principal investigator Dianna Willis, Burke’s director of pain research.

The survival motor neuron protein allows muscles to move, she said. But motor neuron cells are susceptible to dying, and when that happens muscles weaken and atrophy.

The team will study the protein’s role in cell maintenance and try to pinpoint why motor neurons are vulnerable to losing the protein. Their hope is that their findings will point to new therapies that can protect motor neurons from degenerating.

About one in every 10,000 infants have spinal muscular atrophy, and most of them die before age 2, Willis said. Patients with a less severe form of the disease can live into adulthood.

She described the funding as a jump-start grant that will generate preliminary data for a larger grant and long-term research.

The Burke Institute is also awaiting word on another $475,000 grant from the same federal institute, for research on a device that helps people with brain injuries improve their vision.

Sometimes patients with severe brain injuries cannot communicate what they see. The device displays pictures moving across a screen. When the patient tracks the image correctly, music plays. When they stop tracking the image, the music stops.

The device allows doctors to understand what the patient perceives, and it stimulates and improves the patient’s vision.

U.S. Rep. Nita Lowey, D-17th District, announced the grant prematurely on July 14. A Burke spokeswoman said the grant request is still pending.

Burke Medical Research Institute was created in 1978 to look for new strategies for treating patients with spinal cord injuries, stroke and other cognitive and motor disabilities. It is affiliated with Burke Rehabilitation Hospital, with which it shares a campus in White Plains, and with Weill Cornell Medical College.

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