Saving the brain's white matter with mutated mice

Aug 17, 2010

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 to introduce the mutation.

The is made up of two components: grey matter, or nerves, and , 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 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 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 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.

Explore further: New insight that 'mega' cells control the growth of blood-producing cells

add to favorites email to friend print save as pdf

Related Stories

Nerves under control

May 12, 2010

The proper transmission of nerve signals along body nerves requires an insulation layer, named myelin sheath. To be efficient this sheath is designed to have a certain thickness and Swiss researchers from ...

Researchers discover gene crucial for nerve cell insulation

Apr 16, 2007

Researchers funded by the National Institutes of Health have discovered how a defect in a single master gene disrupts the process by which several genes interact to create myelin, a fatty coating that covers nerve cells and ...

Recommended for you

Head injury causes the immune system to attack the brain

2 hours ago

Scientists have uncovered a surprising way to reduce the brain damage caused by head injuries - stopping the body's immune system from killing brain cells. The study, published in the open access journal Acta Neuropathologica Co ...

User comments : 0