ALS Canada-funded research continuing to turn up positive results
MARKHAM, ON, June 4, 2013 /CNW/ - Canadian researcher Edor Kabashi of
the Université de Montréal has developed the first animal model to
study the function of a gene responsible for the highest percentage of
ALS, commonly known as Lou Gehrig's disease. Results to date shed light
on the cause of the devastating disease.
"Our results indicate that normal levels of a gene called C9ORF72 are
crucial for the proper motor function of zebrafish, and loss of its
function causes neurodegeneration," explains Dr. Kabashi.
This is vital information, as ALS is caused by the death of motor
neurons connecting the brain to the muscles. While the specific causes
of ALS have eluded scientists for years, the announcement from Dr.
Kabashi represents an important step forward, in hopes of finding new
therapies and someday, a cure.
"Canadian researchers are recognized internationally for discoveries
that further our understanding of ALS," said Lindee David, CEO, ALS
Canada. "It is a promising time for the ALS community. We are proud to
support their work and are excited by Dr. Kabashi's findings."
First ever published animal model to study the function of gene C9ORF72
In 2011, two international consortia identified that mutations in
C9ORF72 cause a higher percentage of familial and sporadic ALS than any
other gene previously discovered. It was determined that the mutation
makes up approximately one-third of all familial cases of ALS.
Following the important discovery, Dr. Kabashi and Dr. Pierre Drapeau,
also of Université de Montréal, were co-awarded the ALS Canada-funded
Bernice Ramsay Discovery Grant to create zebrafish models of reduced
and mutated C9ORF72. The purpose was to determine the gene's role in
motor function and ability to cause neurodegeneration. Initial results
from the research have recently been accepted for publication in the
prestigious journal Annals of Neurology.
"To validate whether normal levels and/or function of C9ORF72 is
essential for motor neuron health and activity, we developed an animal
model to observe disease presentation in zebrafish," said Dr. Kabashi
"We observed clear motor dysfunction, swimming defects, and
abnormalities at the cellular level."
"Our hope is that these new animal models of ALS will help identify new
avenues for therapy. Many more cellular and animal models of C9ORF72
are currently being studied and we look forward to continued exciting
discoveries in what promises to be another breakthrough year for ALS
research." added David.
ALS is a terminal disease characterized by progressive paralysis of
muscles throughout the body. Ninety percent of ALS patients die within
five years of diagnosis and some in less than one. An estimated 3,000
Canadians have the disease, yet there are currently no effective
treatment options. ALS is caused by death of motor neurons, which
connect the brain to the muscles. While the specific cause remains
unknown, promising discoveries in recent years have provided
significant clues that should pave the way for new therapies and an
About ALS Canada
The ALS Society of Canada, founded in 1977, is the only national
voluntary health organization dedicated solely to the fight against ALS
and support for those with ALS. ALS Canada is the leading
not-for-profit organization working nationwide to fund ALS research
and, with the Provincial ALS Societies, working to improve the quality
of life for Canadians affected by ALS.
SOURCE: ALS Canada
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