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!
Seeking structure with metagenome sequences
20.01.2017 | DOE/Joint Genome Institute
Snap, Digest, Respire
20.01.2017 | Albert-Ludwigs-Universität Freiburg im Breisgau
An important step towards a completely new experimental access to quantum physics has been made at University of Konstanz. The team of scientists headed by...
Yersiniae cause severe intestinal infections. Studies using Yersinia pseudotuberculosis as a model organism aim to elucidate the infection mechanisms of these...
Researchers from the University of Hamburg in Germany, in collaboration with colleagues from the University of Aarhus in Denmark, have synthesized a new superconducting material by growing a few layers of an antiferromagnetic transition-metal chalcogenide on a bismuth-based topological insulator, both being non-superconducting materials.
While superconductivity and magnetism are generally believed to be mutually exclusive, surprisingly, in this new material, superconducting correlations...
Laser-driving of semimetals allows creating novel quasiparticle states within condensed matter systems and switching between different states on ultrafast time scales
Studying properties of fundamental particles in condensed matter systems is a promising approach to quantum field theory. Quasiparticles offer the opportunity...
Among the general public, solar thermal energy is currently associated with dark blue, rectangular collectors on building roofs. Technologies are needed for aesthetically high quality architecture which offer the architect more room for manoeuvre when it comes to low- and plus-energy buildings. With the “ArKol” project, researchers at Fraunhofer ISE together with partners are currently developing two façade collectors for solar thermal energy generation, which permit a high degree of design flexibility: a strip collector for opaque façade sections and a solar thermal blind for transparent sections. The current state of the two developments will be presented at the BAU 2017 trade fair.
As part of the “ArKol – development of architecturally highly integrated façade collectors with heat pipes” project, Fraunhofer ISE together with its partners...
19.01.2017 | Event News
10.01.2017 | Event News
09.01.2017 | Event News
20.01.2017 | Life Sciences
20.01.2017 | Physics and Astronomy
20.01.2017 | Materials Sciences