June – ALS Awareness Month
What is ALS?
Amyotrophic Lateral Sclerosis
(ALS) or more commonly, Lou Gehrig’s disease, is a
neurodegenerative disease in which progressive muscle weakness
leads to paralysis. Most patients die within five years of
diagnosis, but a small percentage survives for ten years or longer.
There is no cure yet. About 3,000 Canadian adults have ALS and less
than 1 in 10 patients has a family history of the disease.Ìý
ALS is a result of the death of motor neurons-nerve cells in the
brain and spinal cord that control voluntary muscle movement.
ALS research and treatment at the Neuro
The Montreal Neurological Institute and Hospital - the Neuro,
provides ALS patients and their families with the finest clinical
care and offers state-of-the-art facilities for basic and clinical
research.Ìý
The ALS clinic at the Neuro is considered a model for multidisciplinary clinical care. Neurologist Dr. Angela Genge, directs the ALS clinical research program, which develops and tests new ALS therapies in collaboration with the Canadian ALS Clinical Trial Consortium (CALS), and with researchers abroad. The Neuro forges local, national and international partnerships in order to advance research into the causes of ALS and to develop effective treatments.Ìý Among the clinical trials undertaken at the clinic is one for the experimental drug, ceftriaxone.Ìý Dr. Genge was honored with the YWCA Woman of Distinction Award in Science and Technology 2011 for her exceptional contributions to patient care and innovative research. This year, she and another ALS researcher at the Neuro, Dr. Heather Durham, were among the distinguished Canadians to receive a Queen Elizabeth II Diamond Jubilee Medal.
ALS researchers at the Neuro
Dr. Heather
Durham has devoted her research to ALS for more than 20
years.Ìý Dr. Durham specializes in developing tissue culture
models of the disease. She seeks to understand why motor neurons
are particularly vulnerable to damage in ALS, and how these neurons
are affected by different genetic mutations responsible for
familial forms of ALS.Ìý She identifies commonalities and
differences in how toxic mutant proteins cause the death of motor
neurons.Ìý Her work has the potential to improve the
preclinical identification and evaluation of therapies.Ìý A
team led by Dr. Durham has uncovered similarities in the two types
of ALS, familial and sporadic.Ìý The findings relate to a
system that plays an important role in maintaining protein quality
control, known as the ubiquitin-proteasome system. This system is
known to be compromised in familial ALS.Ìý The team wanted to
know whether the same system is impaired in sporadic ALS.Ìý The
results using animal models, conclude that there is inadequate
proteasome function both familial and sporadic ALS.Ìý Dr.
Durham collaborates with investigators at the Centre Hospitalier de
l'Université de Montréal (CHUM), Western University, and the Centre
de Recherche de l'Institut du Cerveau et de la Moelle epinière,
INSERM.Ìý Dr. Durham also works to promote ALS research
initiatives across Canada.Ìý She serves as a Board member of
the ALS Society of Canada and as Chair of the Research Policy
Committee.
Dr. Peter McPherson’s research is aimed at understanding the function of proteins in nerve cells using biochemical, molecular, structural and cellular approaches.Ìý His laboratory has identified proteins that could play a fundamental role in neurodegenerative and motor neuron diseases.Ìý Dr. McPherson is also working on the basic mechanisms underlying hereditary spastic paraplegias (HSPs), a genetically diverse group of motor neuron diseases related to ALS.
Dr. Stefano Stifani studies the generation, muscle connectivity, and regeneration of nerve cells used for mastication, swallowing, and motor activities.Ìý His research team has helped to explain the mechanisms underlying the development of those particular types of neurons.Ìý Dr. Stifani’s program aims to provide strategies to manipulate neural stem cells and design approaches that might promote the adult nervous system's ability to repair itself in response to trauma or disease.Ìý
Dr. Eric Shoubridge investigates genes that underlie mitochondrial dysfunction.Ìý Such dysfunction is believed to contribute to many neurodegenerative diseases, including ALS.ÌýÌý His research could help explain the causes of motor neuron diseases.
Dr. Heidi McBride is an internationally recognized expert on mitochondrial biology, the study of how mitochondria provide energy to cells, particularly to muscle cells.Ìý
Dr. Hiroshi Tsuda, studies experimental models of ALS.Ìý These models help us to understand what makes nerve cells susceptible to damage in ALS and in other motor neuron diseases, as well as to allow researchers to test experimental therapies.
ALS and Community Support
The Tony
Proudfoot Fund at the Neuro supports young neuroscientists at the
masters, doctoral and postdoctoral level who undertake research
projects focusing on ALS.Ìý The Fund was established by the
late Tony Proudfoot, former quarterback of the Montreal Alouettes
of the Canadian Football League, after he was diagnosed with ALS in
2007.Ìý
Established in 1983, the ALS Society of Quebec provides support for people with ALS and their families, creates public awareness, and raises funds for patient services and research.Ìý The ALS Society of Quebec works in partnership with other provincial ALS agencies and with the ALS Society of Canada.
In June, ALS Awareness Month, people across Canada raise awareness for ALS, raise funds and don the blue cornflower – the national emblem of ALS in Canada.
About The Neuro
The Montreal Neurological
Institute and Hospital — the Neuro, is a unique academic medical
centre dedicated to neuroscience. Founded in 1934 by the renowned
Dr. Wilder Penfield, the Neuro is recognized internationally for
integrating research, compassionate patient care and advanced
training, all key to advances in science and medicine. The Neuro is
a research and teaching institute of ÎÛÎÛ²ÝÝ®ÊÓƵ University and forms
the basis for the Neuroscience Mission of the ÎÛÎÛ²ÝÝ®ÊÓƵ University
Health Centre.Ìý Neuro researchers are world leaders in
cellular and molecular neuroscience, brain imaging, cognitive
neuroscience and the study and treatment of epilepsy, multiple
sclerosis and neuromuscular disorders. For more information, visit
theneuro.com.
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