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.
Link : www.vrr.ulaval.ca/bd/chercheur/fiche/341072.html
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