New Research Grant for Dr. Jean-Pierre Julien Dr. Jean-Pierre Julien, an ALS Society of Canada board member and a prominent ALS researcher at Laval University Research Centre in Quebec has been awarded a research grant from the the ALS Association in the United States. “Role of Chromogranin-mediated Secretion of Superoxide Dismutase Mutants in ALS Pathogenesis,” is the title of the research grant. The ALS Society of Canada agreed to co-fund the grant with the ALS Association in the United States after the grant was made.
A new suspect in ALS prompts Julien and colleagues to delve further into the role played by chromogranins. These components of certain nerve cells appear to associate with the mutated ALS enzyme, SOD1. New ALS mutant mice will be made that make more than normal amounts of chromogranins, to see if these mice will show worsened disease. The cellular protectors called chaperones may also have roles in ALS. Funding of this project is in partnership with The ALS Society of Canada.
What are chromoganins?
Chromogranins are molecules made by nerve cells that usually aid production and packaging of other proteins. Investigators working with Julien have discovered that chromogranins are binding to mutant SOD1, the protein linked to inherited forms of amyotrophic lateral sclerosis (ALS). The two proteins appear together in spinal cord of mice with mutant SOD1. Cells grown in laboratory dishes that produce more than the usual amount of chromogranins also secrete mutant SOD1.
Julien and his team have found that when mutant SOD1 is secreted in cell culture, an inflammatory response is produced in microglia cells, and motor neurons die.
The investigators will find out if chromogranins are important to this toxicity of mutant SOD1. They will induce increased production of chromogranins, in the spinal motor neurons of mice bearing the SOD1 mutation, and see if this exacerbates their disease. Also, they will suppress chromogranin production, to see if secretion of mutant SOD1 is suppressed, and whether this prevents the pathologic changes.
For more information on Professor Julien’s work please refer to our research publication the Northern Neuron which can be downloaded http://www.als.ca/_researchers.aspx
| Posted On: Tuesday, February 22, 2005 Modified: Tuesday, February 22, 2005 Category: Researchers Posted By:
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