Vanishing White Matter (VWM) disease is a devastating condition that involves the destruction of brain myelin due to a mutation in a central factor.
To understand the disease and test potential treatments that could apply to other disorders, such as multiple sclerosis, Prof. Orna Elroy-Stein of Tel Aviv University's Department of Cell Research and Immunology is leading a scientific breakthrough by developing laboratory mice which carry the VWM mutation — an important new tool.
The mice harbor a mutation of the eIF2B enzyme, which regulates protein synthesis in every cell throughout the body. The key to the new development, says Prof. Elroy-Stein, was the use of genetically-engineered embryonic stem cells to introduce the mutation.
The brain is made up of two components: grey matter, or nerves, and white matter, or glial cells which support the nerves and produce myelin, which wraps around and protects nerve extensions. Recently described in the prestigious journal Brain, the creation of these mutated mice allows for new research on VWM diseases, which trigger loss of myelin in the brain, leading to paralysis and possible death.
A new breed of mouse and man
Until now, VWM researchers did not have a comparable animal model with which to study the disease. Now, for the first time, researchers can use a living organism to follow the exact process of myelin destruction, providing valuable information about the molecular mechanisms of the disease, which are currently unknown.
The big surprise in this discovery, she says, is that the gene mutation impacts only the white matter of the brain, rather than causing disease throughout the body. This will allow researchers to learn about myelin formation and maintenance.
"For the first time ever, we can follow the regulated expression of the protein components of myelin," explains Prof. Elroy-Stein, whose techniques include magnetic resonance imaging to investigate what's happening in the brains of the mice.
Not only will this discovery lead to a greater understanding of diseases that affect the brain's white matter, the mice are also an invaluable testing ground for new treatments. But according to Prof. Elroy-Stein, understanding the disease is the first step. "In order to develop effective therapies, one has to understand the mechanisms," she says.
Prof. Elroy-Stein notes that this is an entirely TAU-led project. The team of researchers include her Ph.D. students Michal Geva, Yuval Cabilly, Liraz Marom, Nina Mindroul, and Gali Raini; lab technician Dalia Pinchasi; and Dr. Yaniv Assaf of Tel Aviv University's Department of Neurobiology.
Keep up with the latest AFTAU news on Twitter: http://www.twitter.com/AFTAUnews
George Hunka | EurekAlert!
Nanoparticle Exposure Can Awaken Dormant Viruses in the Lungs
16.01.2017 | Helmholtz Zentrum München - Deutsches Forschungszentrum für Gesundheit und Umwelt
Cholera bacteria infect more effectively with a simple twist of shape
13.01.2017 | Princeton University
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...
At TU Wien, an alternative for resource intensive formwork for the construction of concrete domes was developed. It is now used in a test dome for the Austrian Federal Railways Infrastructure (ÖBB Infrastruktur).
Concrete shells are efficient structures, but not very resource efficient. The formwork for the construction of concrete domes alone requires a high amount of...
Many pathogens use certain sugar compounds from their host to help conceal themselves against the immune system. Scientists at the University of Bonn have now, in cooperation with researchers at the University of York in the United Kingdom, analyzed the dynamics of a bacterial molecule that is involved in this process. They demonstrate that the protein grabs onto the sugar molecule with a Pac Man-like chewing motion and holds it until it can be used. Their results could help design therapeutics that could make the protein poorer at grabbing and holding and hence compromise the pathogen in the host. The study has now been published in “Biophysical Journal”.
The cells of the mouth, nose and intestinal mucosa produce large quantities of a chemical called sialic acid. Many bacteria possess a special transport system...
10.01.2017 | Event News
09.01.2017 | Event News
05.01.2017 | Event News
17.01.2017 | Earth Sciences
17.01.2017 | Materials Sciences
17.01.2017 | Architecture and Construction