LAVAL UNIVERSITY RESEARCHER AWARDED $345K MDA GRANT TO UNDERSTAND WHY ONE MOLECULE HASTENS ALS ONSET BY 10 YEARS
TUCSON, Ariz., Aug. 18, 2010 – Jean Pierre Julien, Ph.D., of Laval University in Quebec, Canada, is one of the world’s foremost researchers in amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease). The devastating neurodegenerative disease targets motor neurons in the brain and spinal cord, causing paralysis and death, usually within five years of diagnosis.
 | | Jean Pierre Julien |
Julien has been awarded a new $345,000 research grant by the Muscular Dystrophy Association to find out how a single molecule that his lab recently discovered increases susceptibility to ALS and hastens disease onset by 10 years. MDA is the world’s largest non-governmental funder of ALS research.
Molecules known as chromogranins are made by nerve cells and can bind to certain proteins including mutant, misfolded forms of SOD1, a protein known to cause ALS. Julien found that one variant of chromogranin B is detected in a disproportionately high number of ALS patients and therefore it constitutes a risk factor for ALS. What’s more, Julien’s team discovered that ALS patients with this chromogranin B variant develop signs of the disease, on average, almost 10 years sooner than those without this variant.
“Dr. Julien is a giant in the ALS field,” said Stanley H. Appel, M.D., chairman of the MDA Medical Advisory Committee and himself an ALS research pioneer. “His discovery that misfolded SOD1 is secreted from motor neurons bound to chromogranins was a landmark contribution and suggests that variations in chromogranin itself can be a risk factor in ALS.
“His new funding from MDA will allow his research team to unlock the link between chromogranins and susceptibility to ALS,” explained Dr. Appel. “Once we understand this connection, we may be able to develop new therapies specifically aimed at these toxic secretions.”
Julien is one of 38 research leaders awarded $14.1 in new multi-year grants by MDA’s Board of Directors at its July meeting.
Research grant applications are peer-reviewed twice yearly by MDA’s Medical and Scientific Advisory Committees, comprising world-renowned experts in neuromuscular disease research. The most promising of some 500 applications received each year are recommended for funding to the MDA Board of Directors.
According to R. Rodney Howell, M.D., chairman of the MDA Board of Directors, “Generous Americans responding to the annual Jerry Lewis MDA Telethon, and to thousands of other special events benefiting the families served by MDA, deserve much of the credit for the rapid progress being made towards treatments for neuromuscular diseases. It’s their strong belief in MDA’s capable stewardship of public funds that’s enabling so much to be accomplished – even in a sluggish economy.”
Often credited for its leadership in building the field of neuromuscular disease research, MDA has also enhanced clinical care for individuals affected by muscle disorders, achieving important quality of life and longevity gains. The Association, which annually invests almost $39 million on research worldwide, is the first nonprofit to earn a Lifetime Achievement Award from the American Medical Association (“for significant and lasting contributions to the health and welfare of humanity”).
MDA-funded scientists have uncovered the genetic defects that cause several forms of muscular dystrophy; Charcot-Marie-Tooth disease (CMT); a form of amyotrophic lateral sclerosis (ALS or Lou Gehrig’s disease); childhood spinal muscular atrophy (SMA) and other neuromuscular conditions. Now entering a period of increasing numbers of clinical trials of potential therapeutics, the Association’s network of approximately 200 hospital-affiliated clinics is instrumental in identifying appropriate candidates for clinical trials, and in helping to refine outcome measures for those clinical trials.
More than 1 million people in America are affected by neuromuscular diseases.
MDA is the nonprofit health agency dedicated to curing muscular dystrophy, ALS and related diseases by funding worldwide research (view MDA research at http://www.mda.org/research/gaag/2010/b-roll.html). The Association also provides comprehensive health care and support services, advocacy and education.
For more information about these new grants, visit MDA's "Grants at a Glance,” an online slideshow that showcases each grant with photos and detailed information |
