A protein that promotes the growth of neurons and blood vessels appears to stop the progression of a genetic disease that causes degeneration of the cerebellum, according to new preclinical Northwestern Medicine research published in Nature Medicine.
The disease, spinocerebellar ataxia type 1, typically strikes people in their 30s and 40s and causes degeneration of the cerebellum, the part of the brain that helps coordinate movement. As the disease progresses over 10 to 20 years, patients eventually die from aspiration or infectious pneumonia.
The disease is caused by a mutation in a protein called ataxin-1, which plays a role in regulating a protein called vascular endothelial growth factor or VEGF. When Northwestern scientists replenished VEGF in the brains of a mouse model of this disease, the brains -- which had showed atrophy in the cerebellum -- began to appear more normal, with an increase in connections between neurons. The mice also had improved balance.
"If you give VEGF early in the disease, you prevent degeneration later in life," said Puneet Opal, M.D., associate professor of neurology and of cell and molecular biology at Northwestern University Feinberg School of Medicine and a neurologist at Northwestern Memorial Hospital, who also treats ataxic patients. "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."
The study also provides a new understanding of the degenerative disease. Because patients are born with the mutation for the disease but don't show signs of it until midlife, Opal said that indicates the aging process appears to play a role in development of the disease.
"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."
Other authors of the paper include first author Marija Cvetanovic, research assistant professor of neurology, and Jay Patel, a former undergraduate student, who both are from Northwestern and work in the Opal lab, and Ameet R. Kini and Hugo Marti from Loyola University Chicago Stritch School of Medicine and the University of Heidelberg, respectively.
Megan Fellman | EurekAlert!
'Y' a protein unicorn might matter in glaucoma
23.10.2017 | Georgia Institute of Technology
Microfluidics probe 'cholesterol' of the oil industry
23.10.2017 | Rice University
Salmonellae are dangerous pathogens that enter the body via contaminated food and can cause severe infections. But these bacteria are also known to target...
University of Maryland researchers contribute to historic detection of gravitational waves and light created by event
On August 17, 2017, at 12:41:04 UTC, scientists made the first direct observation of a merger between two neutron stars--the dense, collapsed cores that remain...
Seven new papers describe the first-ever detection of light from a gravitational wave source. The event, caused by two neutron stars colliding and merging together, was dubbed GW170817 because it sent ripples through space-time that reached Earth on 2017 August 17. Around the world, hundreds of excited astronomers mobilized quickly and were able to observe the event using numerous telescopes, providing a wealth of new data.
Previous detections of gravitational waves have all involved the merger of two black holes, a feat that won the 2017 Nobel Prize in Physics earlier this month....
Material defects in end products can quickly result in failures in many areas of industry, and have a massive impact on the safe use of their products. This is why, in the field of quality assurance, intelligent, nondestructive sensor systems play a key role. They allow testing components and parts in a rapid and cost-efficient manner without destroying the actual product or changing its surface. Experts from the Fraunhofer IZFP in Saarbrücken will be presenting two exhibits at the Blechexpo in Stuttgart from 7–10 November 2017 that allow fast, reliable, and automated characterization of materials and detection of defects (Hall 5, Booth 5306).
When quality testing uses time-consuming destructive test methods, it can result in enormous costs due to damaging or destroying the products. And given that...
Using a new cooling technique MPQ scientists succeed at observing collisions in a dense beam of cold and slow dipolar molecules.
How do chemical reactions proceed at extremely low temperatures? The answer requires the investigation of molecular samples that are cold, dense, and slow at...
23.10.2017 | Event News
17.10.2017 | Event News
10.10.2017 | Event News
23.10.2017 | Life Sciences
23.10.2017 | Physics and Astronomy
23.10.2017 | Health and Medicine