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Saving the Brain's
White Matter with Mutated Mice
August, 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
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.
