Fondation André-Delambre - ALS
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"A spark that lights the way toward a solution"

-André Delambre
Scientifique Research Committee
Scientifique Research Committee

Jean-Pierre Julien
Jean-Pierre Julien
Canada Research Chair in the Mechanisms of Neurodegeneration

Laval University
Tier 1 - September 1, 2003
(514) 934-1934, ext. 42361

(514) 934-1934, ext.

Research Involves

Studying the molecular mechanisms of neurodegeneration caused by cytoskeletal anomalies and by genetic mutations associated with amyotrophic lateral sclerosis.

Research Relevance

New targets for therapy of, and new tests of medications for, neurodegenerative diseases.

Research on Neurodegeneration

Amyotrophic lateral sclerosis (ALS) is a disease in adults characterized by selective degeneration of the motor neurons (the nerve cells that control muscular contraction). This disease leads to progressive weakening of the skeletal muscles, paralysis and death. We have known since 1993 that mutations in the superoxide dismutase (SOD1) gene are responsible for 20 per cent of the cases of ALS in families. However, in the majority of cases, the causes of the disease remain unknown. It is thought that multiple genetic factors are probably implicated in these “sporadic” cases of ALS. Some recent studies suggest that abnormal accumulations of the proteins that form the cytoskeleton may contribute to the process of neurodegeneration that characterizes this disease. However, the toxic processes associated with mutated SOD1 or with abnormalities of the cytoskeleton are still poorly understood and at present we have no therapeutic approach that can halt the progress of the disease.

Holder of a doctorate in biochemistry and possessing special expertise in neurobiology, as Canada Research Chair in the Mechanisms of Neurodegeneration, Jean-Pierre Julien will devote most his time to studying the mechanisms implicated in the selective loss of motor neurons. To do this, he will use rats with ALS bred in his laboratory. One aspect of his research seeks to better understand the deleterious effects that occur when neurofilaments accumulate on intracellular transports. Prof. Julien will also study the role of inflammation in the pathogenesis of ALS. In addition, he will use rats afflicted with the juvenile form of ALS—recently developed in his laboratory—to determine other factors that are linked to the degeneration and vulnerability of certain types of motor neurons. While we know very well that ALS is a complex disease, Prof. Julien’s laboratory recently demonstrated that a combination of three medications, each with a different therapeutic target, was able to significantly impede the progress of ALS in laboratory rats. This discovery will make it possible to soon develop clinical trials of this pharmacological “cocktail” for human patients suffering from ALS.

Prof. Julien seeks to discover and demonstrate the molecular and cellular mechanisms of ALS that contribute to the loss of motor neurons. His research will have important implications for the discovery of new therapeutic targets and for the development of more effective treatments for neurodegenerative diseases.


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