ALS Research Synopsis

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Summary of ALS Research in Canada

The ALS Society of Canada is committed to:

Investing our research funds where they will have the most impact.
Funding excellent and relevant peer-reviewed research.
Funding research that is evaluated at a high level using international evaluation methods adopted by the Canadian Institutes of Health Research.

We fund only the most promising projects by the finest scientists and, over the years, this strategy has been rewarded with significant advances in our knowledge of ALS. With the acceleration of results due to advances in neurology and other areas of science, we know that, effective therapies and a cure are now, more than ever, within reach. The following is a list of researchers who are currently studying ALS and neuromuscular diseases in Canada.

Research Projects funded by the ALS Society and/or the Neuromuscular Research Partnership

Neuromuscular Research Partnership
In 1999, The ALS Society of Canada partnered with the Canadian Institutes of Health Research, and Muscular Dystrophy Canada to create the Neuromuscular Research Partnership. This partnership was created to collectively fund health research by providing operating grants in the area of neuromuscular diseases with a mandate to find a cause, treatment options and eventually result in a cure.

Dr. Jean-Pierre Julien Canada Research Chair, Mechanisms of Neurodegeneration
Where:	Université Laval
Project: Funding: Project: Funding: Project: Funding: Project: Funding: Project: Funding:	Role of Chromogranin-mediated Secretion of Superoxide Dismutase Mutants in ALS Pathogenesis. Chromogranins are molecules made by nerve cells that usually aid production and packaging of other proteins. CIHR, NRP, The ALS Association (U.S.), The Robert Packard Center for ALS Research at Johns Hopkins, and Réseau de Recherche in Transgénèse du Québec Pathogenic mechanisms associated with neurofilament disorganization NRP (2005) - Five years The role of inflammation in pathogenesis of ALS (with Dr. Serge Rivest) NRP (2003)-Five years Generation and analysis of a new mouse model for ALS NRP (2002)-One year The role of inflammatory cytokines in pathogenesis of ALS NRP (2000)-Three years

Dr. George Karpati & Dr. Joséphine Nalbantoglu
Where:	McGill University, Montreal Neurological Institute
Project: Funding: Project: Funding: Project: Funding: Project: Funding:	Molecular therapies for dystrophin deficiency NRP (2005) - Three years Extrasynaptic endogenous utrophin upregulation in skeletal muscle: A therapeutic approach for Duchenne muscular dystrophy (DMD) NRP (2004)-Three years (George Karpati) Development of efficient and safe gene transfer to skeletal muscle for the therapy of dystrophin deficiency NRP (2002)-Three years (George Karpati & Josephine Nalbantoglu) Utrophin upregulation in skeletal muscle: A Therapeutic Approach for DMD NRP (2000) – Three years
Overview:	Exploring how utrophin, a protein, can be “upregulated” or inserted to lessen the impact of a dystrophin deficiency. DMD is caused by a deficiency of the dystrophin protein.

Dr. David J. Picketts
Where:	Ottawa Health Research Institute
Project:	Genetic dissection of ISWI function during neurogenesis
Overview:	This project will help to define how stem cells work and what steps are required to direct a stem cell to become a muscle or nerve cell.
Funding:	NRP (2005)- Three years

Dr. Michael Sinnreich
Where: Project: Funding:	McGill University, Montreal Neurological Institute Development of therapeutic strategies for dysferlin deficiency NRP (2007) - Three years
Project:	Modular flexibility of dysferlin-possible applications for gene therapeutic strategies
Overview:	This project will contribute to the further understanding of dysferlinopathies and will provide clues for future genetic therapies.
Funding:	NRP (2005) – One year

Dr. Kenneth E. Hastings
Where:	Montreal Neurological Institute, McGill University
Project:	Fiber-type-specific and actively-regulated gene expression in fast skeletal muscle
Overview:	Investigating the molecular events that determine muscle fiber-type. Muscle fiber is an important aspect of muscle function. Muscle fibres develop into their two separate varieties – fast and slow.
Funding:	NRP (2005) - Three years, NRP (2002)-Three years

Dr. Hakima Moukhles
Where:	University of British Columbia
Project:	Dystroglycan function in glial cells
Overview:	Forthcoming
Funding:	NRP (2005)- Three years

Dr. Louise R. Simard
Where:	Hôpital Sainte-Justine( Montreal)
Project:	Characterization of "survival of motor neurons" (SMN) gene regulation
Overview:	Studying how SMN gene expression is regulated during development. Mutations in the SMN gene are responsible for childhood spinal muscular atrophy, a severe lower motor neuron disease.
Funding:	NRP (2005) - Three years, NRP (2001) - Three years

Dr. Heather Durham
Where:	McGill University, Montreal Neurological Institute
Project:	The role of protein chaperones and proteasome-mediated proteolysis in the pathogenesis of motor neuron diseases
Overview:	The Durham lab studies why motor neurons are more vulnerable to damage in ALS than other cells in order to identify protective therapies. Durham’s lab is investigating the importance of protein chaperones and proteosomes in helping cells deal with damaged proteins. The chaperones’ job is to round up damaged proteins (in this case, the mutant SOD1) and shuttle them to the proteosomes where they are chopped up and discarded.
Funding:	NRP (2005)-Five years, The ALS Association (U.S.), and the Muscular Dystrophy Association (U.S.)

Dr. Bernard Brais
Where:	Centre Hosp. de l’Université de Montreal
Project: Funding: Project: Funding:	Cloning and characterising the mutated gene responsible for a new form of French-Canadian recessive spastic ataxia NRP (2005) - Three years Cloning and characterising the mutated gene responsible for a French-Canadian recessive ataxia associated with severe polyneuropathy NRP (2003) - Three years
Overview: Project: Funding:	Seeking to understand how the mutation – a lengthening of a portion of specific protein – leads to OPMD and other related disorders so that further research can be directed toward effective therapies. OPMD is a hereditary disease that results in generalized muscle weakness, particularly those responsible for eyelid elevation and swallowing. Oculopharyngeal muscular dystrophy (OPMD) and polyalanine toxicity NRP (2001)-Three years

Dr. F.J. Dilworth
Where:	Ottawa Health Research Institute
Project:	Elucidating the mechanisms directing temporally ordered gene expression by myoD
Overview:	The specific aim of this project is to contribute to refining the stem cell research that holds significant hope for future treatments of many forms of muscular dystrophy.
Funding:	NRP (2005) - Five years

Dr. Edor Kabashi
Where: Project: Funding:	University of Montreal Developing and characterizing novel models of ALS and other neurological disorders in zebrafish Tim E. Noël Fellowship in ALS Research (2006) - Three years
Project:	Problems with Protein Disposal in ALS
Overview:	Contributing to a better understanding of pathophysiology of ALS and other neurodegenerative disorders and to offer new therapeutical agents to help people with ALS.
Funding:	Awarded two years of funding by the ALS Society of Canada (2004)

Miranda Tradewell, PhD Student
Where:	McGill University, Montreal Neurological Institute (Dr. Heather Durham’s lab)
Project:	The Role of Calcium in Motor Neuron Disease
Overview:	Seeking not only to examine the cause of motor neuron diseases such as ALS, but to gain a better understanding of why the biology of motor neurons makes them vulnerable to the toxicity of mutant proteins compared to other cells.
Funding:	Awarded three years of funding by the ALS Society of Canada (2004)

Dr. Avijit Chakrabartty
Where: Project: Funding:	University Health Network Ontario Cancer Institute Interplay of oxidative stress and protein misfolding in ALS NRP (2007) - Three years
Project:	Protein misfolding and conformational disease
Overview:	Studying how protein folding affects cells and the entire body to better understand ALS.
Funding:	NRP (2004) – Three Years

Dr. Robin J. Parks
Where:	Ottawa Health Research Institute
Project:	Adenovirus vectors for gene therapy of muscle
Overview:	Has several advantages over traditional Ad vectors and has shown promising results in pre-clinical studies.
Funding:	NRP (2004)-Three years (with Jonathan L. Bramson) NRP (2001)-Three years

Dr. Vanessa J. Auld
Where:	University of British Columbia
Project:	Glial cell development and function at the Drosophila neuromuscular junction
Overview:	Studying the physiological basics of glia cells in fruit fly development. Nervous system development and function is guided by control cells called glia.
Funding:	NRP (2004)-Three years

Dr. Jérôme Frenette
Where:	Université Laval
Project:	Inflammatory cell recruitment and function in skeletal muscles following hind limb unloading and reloading: New strategies to prevent muscle atrophy and dysfunction
Overview:	Studying how a side effect of mobility – secondary atrophy – can be reduced or diminished.
Funding:	NRP (2004)-Three years

Dr. Jiming Kong
Where: Project: Funding:	University of Manitoba ALS: Rescue of mutant SOD1-induced motor neuron death by targeting the BNIP3 death gene family NRP (2007) - One year
Project:	ALS: Role of BNIP3 in mutant SOD1-induced motor neuron death
Overview:	Examining ways to slow or decrease ALS symptoms. BNIP3 is a recently discovered pro-death protein which appears to induce apoptosis in motor neurons. Apoptosis, or programmed cell death, is a genetically controlled mechanism for the body to eliminate cells that have outlived their usefulness.
Funding:	NRP (2004)-Three years

Dr. Susan O. Meakin
Where:	John P. Robarts Research Institute (London)
Project:	Nesca, a novel intracellular signaling adapter facilitates neurotrophin dependent neurite outgrowth.
Overview:	Determining the mechanism(s) which facilitate a novel intracellular protein that Meakin and her group have identified, named Nesca -- new molecule containing an SH3 domain at the carboxyterminus.
Funding:	NRP, CIHR, and the National Cancer Institute of Canada

Dr. Robert G. Korneluk
Where:	Children’s Hospital of Eastern Ontario (Ottawa)
Project:	The X-linked inhibitor of apoptosis (XIAP): A therapeutic agent for the treatments of muscular dystrophy
Overview:	Helping to develop therapies for neuromuscular disorders. Apoptosis – or programmed cell death – is a genetically controlled mechanism for the body to eliminate cells that have outlived their usefulness.
Funding:	NRP (2004) - Three years

Dr. Janice Robertson Canada Research Chair, Molecular Mechanisms of ALS
Where:	The Centre for Research in Neurodegenerative Disease at the University of Toronto
Project:	Peripherin abnormalities in ALS
Overview:	Focusing on peripherin, a key protein involved in the pathological aggregates found in motor neurons in ALS. Peripherin is found in the neurofilament aggregates that clog motor neurons in ALS. By searching for toxic mutations in peripherin, she hopes to discover the mechanisms that cause ALS. Robertson is the only researcher in Toronto who is focused exclusively on ALS.
Funding:	NRP, Motor Neurone Disease Association (U.K.), Muscular Dystrophy Canada (2006) and The ALS Association (U.S.)

Janka Hegedus, PhD Student
Where:	University of Edmonton, Alberta. Works in Dr. Tessa Gordon’s lab
Project:	Thesis work involves characterizing the progressive loss of motoneurons and their muscle fiber connections in ALS.
Funding:	The NRP, National Sciences and Engineering Research Council and the Alberta Heritage Foundation for Medical Research.

Dr. Elizabeth M. Meiering
Where:	University of Waterloo
Project:	Folding and aggregation of ALS-associated mutant superoxide dismutases
Overview:	Looking at different classes of mutations and then characterising how the mutants behave and how that may be linked to the disease. Has successfully demonstrated a link between SODI mutations and neuronal aggregates.
Funding:	NRP (2003)-Three years (with James R. Lepock)

Dr. Alexander E. Mackenzie
Where:	Children’s Hospital of Eastern Ontario (Ottawa)
Project:	Modulation of apoptosis in mouse models of spinal muscular atrophy
Overview:	Examining how cell death can be regulated in spinal muscular atrophy.
Funding:	NRP (2003)-Three years (with Nathalie H. Gendron)

Dr. Stefano Stifani
Where: Project: Funding:	McGill University Motor neuron differentiation, connectivity and regeneration NRP (2007) - Four years
Project:	Regulation of neuronal development in the mammalian nervous system
Overview:	Examining how the growth and development of neurons is controlled to better understand their function and develop possible treatments for neuromuscular disorders.
Funding:	NRP (2003) - Five years

Dr. Michael J. Ferns
Where:	The Res. Institute of the McGill University Health Centre
Project: Funding: Project: Funding:	Neurotransmitter receptor localization at the synapse: Regulation by rapsyn NRP (2002) - Three years Agrin’s role in cholinergic synapse formation NRP (2001) - Three years
Overview:	Understanding how a signaling factor – agrin – directs the formation of the cholinergic synapses. Agrin is required for the formation of the neuromuscular junction.

Dr. Tessa Gordon
Where:	University of Alberta
Project:	A possible link between motoneuronal death and sprouting in ALS
Overview:	Investigating the effect of injury, exercise and muscle fibre-type in transgenic mice and human tissue to gain insight into the pathways and progress rates for ALS.
Funding:	Medical Research Council of Canada, Canadian Neurotrauma Research Program, NRP, and the Paralyzed Veterans of America Spinal Cord Research Foundation

Dr. Stephen H. Gee
Where: Project: Funding:	University of Ottawa Investigating the role of diacylglycerol kinasezeta in the assembly and maintenance of the myofibrillar apparatus in skeletal muscle NRP (2007) - Three years
Project:	The role of diacylglycerol kinase-zeta and syntrophins in neurite outgrowth
Overview:	Studying the molecules that interact with dystrophin to better understand cognitive impairment associated with DMD. Syntrophins link receptors, ion channels, and signaling proteins to dystrophin.
Funding:	NRP (2002)-Three years

Dr. Henry J. Klamut
Where:	University Health Network Ontario Cancer Institute
Project:	Telomerase-mediated lifespan extension applied to the development of autologous myoblast transplantation strategies for DMD
Overview:	Hoping to genetically engineer the patient myoblasts (undeveloped muscle cells) to express dystrophin, or utrophin.
Funding:	NRP (2002)-Three years

Dr. Jeffrey T. Henderson
Where:	University of Toronto
Project:	Role of Eph-B family receptors in regulating motoneuron identity and somatotopic axon outgrowth in the murine spinal cord
Overview:	Studying how neurons communicate with each other during development and following injury. Eph receptors are a family of receptor tyrosine kinases – proteins that regulate communication between cells.
Funding:	CIHR, NRP

Dr. Robin N. Michel
Where:	Concordia University
Project:	Calcineurin signaling in the regulation of skeletal muscle fiber
Overview:	Michel was the first to demonstrate calcineurin’s crucial role in the growth of adult muscle. Working to decipher some of the other factors affecting muscle growth. Calcineurin is found in all muscle types.
Funding:	NRP (2002)-Three years, NRP, the Natural Sciences and Engineering Research Council of Canada

Dr. David J. Schreyer
Where:	University of Saskatchewan
Project:	Regulation of neuronal phenotype by a muscle-derived factor
Overview:	Investigating the relationship between muscle cells and the motor neurons that target them, with the hope of identifying the factor that affects the way neurons react to injury.
Funding:	NRP (2002)-Three years

Dr. Margaret Fahnestock & Dr. James R. Bain
Where:	McMaster University
Project:	Mechanism of sensory protection of denervated muscle
Overview:	Understand how sensory protection prevents muscle atrophy and determine if sensory protection is a potentially valuable therapeutic approach to nerve damage following injury or disorders such as muscular dystrophies, spinal muscular atrophies and ALS.
Funding:	NRP (2003)-Three years

Dr. Carl-Eric Aubin
Where:	Hôpital Sainte-Justine (Montreal)
Project:	Study of the biomechanical factors related to the progression of scoliotic deformities in DMD
Overview:	Identify the biomechanical factors involved in the progression of spinal curvature in children with DMD and understand the possible pathological pathways of scoliosis.
Funding:	NRP (2001)-Three years

Dr. Salvatore T. Carbonetto
Where:	Montreal General Hospital Research Institute, McGill University
Project:	Dystrophin associated proteins in synapse structure and function
Overview:	Investigating the dystrophin complex in neurons. DMD results from mutations in the gene for dystrophin.
Funding:	NRP (2001)-Three years

Dr. Denis Gravel
Where:	Université de Montreal Institut de réadaptation de Montreal
Project:	Rôle des contractures dans la limitation de la marche des enfants atteints de dystrophie musculaire de Duchenne
Overview:	Studying the biomechanics of how muscle constriction in DMD limits gait
Funding:	NRP (2001)-Three years

Dr. Bernhard Juurlink
Where:	University of Saskatchewan
Project:	Oxidative stress in the CNS and motoneuron disease
Overview:	Investigating how oxidative stress leads to inflammation, and how cellular mechanisms to slow oxidative stress may be enhanced to delay the onset of ALS. Oxidative stress is particularly associated with the familial form of ALS, caused by a mutation in SOD1.
Funding:	NRP (2001)–Three years

Dr. David H. MacLennan
Where:	University of Toronto
Project:	The pathophysiological and genetic basis for muscle diseases resulting from calcium dysregulation
Overview:	Studying how calcium dysregulation can produce muscle disease.
Funding:	NRP (2001)-Five years

Dr. Jean Mathieu
Where:	Centre Hosp. de l’Université de Montreal, Hôpital de Jonquière
Project:	Consequences of neuromuscular genetic disorders: disabilities, social participation, quality of life and their determinants among individuals with myotonic dystrophy
Overview:	Myotonic dystrophy is the most common form of muscular dystrophy in adults. Attempting to measure all factors, whether genetic, personal or environmental, that could play a role in the subject’s social participation and quality of life.
Funding:	NRP (2001)-Three years

Dr. Gregory M. Ross
Where:	Queen’s University
Project:	Motor neuron death resulting from a zinc-or copper-induced loss of trophic support
Overview:	Conducting basic research to identify critical steps in the nerve cell death process.
Funding:	NRP (2001)-Three years

Dr. Ilona S. Skerjanc
Where:	University of Western Ontario
Project:	The molecular biology of skeletal muscle development
Overview:	Identifying the proteins that are key regulators of muscle development (termed transcription factors) to understand how they function and to determine what controls their ability to function.
Funding:	NRP (2001)-Five years

Dr. Mei Zhen
Where:	Mount Sinai Hospital
Project:	Dissecting molecular mechanisms that regulate the presynaptic differentiation in C. elegans
Overview:	Working on how synaptogenesis is regulated. Neurons establish connections with each other, called synapses, and transmit information from one neuron to another and throughout the body.
Funding:	NRP (2001) - Three years

Dr. Michael J. Strong Recipient of the Sheila Essey Award (2005)
Where:	Robarts Research Institute (London)
Project: Funding: Project: Funding:	Intermediate filament expression in sporadic ALS NRP (2001) - Two years A molecular and neuropathological characterization of the Cognitive impairment of sporadic ALS ALS Canada, The ALS Association (U.S.), Muscular Dystrophy Association, The Scottish Rite Charitable Foundation, Michael Halls Endowment and the CIHR (NET grant, Serge Rivard, lead investigator)
Overview:	Discovering the role of neurofilament aggregates in ALS pathways.

Dr. Charles Krieger
Where:	Simon Fraser University and University of British Columbia
Project: Funding: Project: Funding:	Functional role of hematogenous inflammatory cells in ALS NRP (2004) - Three years (with Fabio M. Rossi) and NSERC Modulation of neuron death by protein kinase C and calcineurin in ALS NRP (2000)-Two years
Overview:	Attempting to find out why nerve cells die in ALS. In previous work he found that those living with ALS have elevated amounts the enzyme, protein kinase C, which has numerous functions within the cells of the body.

Dr. Jack Puymirat
Where:	Centre Hospitalier, Universitaire de Québec
Project:	Ribozymes and antisense RNA as a tool to study myotonic dystrophy
Overview:	Attempting to create gene therapies using "road blocks" that will stop the mutated genes that cause myotonic dystrophy from being read thereby reducing the symptoms of myotonic dystrophy.
Funding:	NRP (2000)-Three years, NRP (2003)-Three years

Dr. Jacques P.Tremblay
Where: Project: Funding:	Université Laval Hospitalier de l’Université Laval Improving MPC transplantation by increasing IGF-1 or MGF stimulation NRP (2007) - Three years
Project: Funding: Project: Funding:	Treatment of DMD: Correction of mutated dystrophin mRNA with ribozymes NRP (2001)-Three years Autotransplantation of genetically modified myoblasts and muscle derived stem cells NRP (2000)-Three years, NRP (2003) - Three years
Overview:	Growing myoblasts for transplantation in humans.

Dr. John Roder
Where:	Mount Sinai Hospital - Samuel Lunenfield Research Institute
Project:	Novel therapies for ALS
Overview:	Exploring the development of effective anti-glutamate agents that may stall or even reverse the relentless progression of ALS.
Funding:	NRP (2000)-Three years

Dr. Anthony Gramolini
Where:	University of Toronto
Project: Funding:	Molecular basis of ryanodine receptor regulation and function in skeletal muscle NRP (2007) - Five years

Dr. Paul Holland & Dr. Joséphine Nalbantoglu
Where:	McGill University, Montreal Neurological Institute
Project: Funding:	Combinatorial use of viral vectors for the gene therapy of muscle NRP (2007) - Two years

Dr. Jasna Kriz
Where:	Université Laval
Project: Funding:	Live imaging and analysis of disease onset and progression in ALS NRP (2007) - Three years

Dr. Tanja Taivassalo
Where:	McGill University
Project: Funding:	Exercise-induced upregulation of mitochondrial gene expression: Therapeutic strategies for mitochondrial disease NRP (2007) - Three years

Dr. Christine Vande Velde
Where:	Université de Montréal/Centre Hosp. de l’Université de Montréal
Project: Funding:	Identification of the mechanisms of motor neuron degeneration in ALS NRP (2007) - Three years

Additional ALS Researchers

Dr. Shirley Liu
Where:	The Centre for Research in Neurodegenerative Diseases at the University of Toronto
Project:	Involves transgenic mice and the SOD1 gene that is responsible for the hereditary form of ALS. Liu is interested in an active immunization approach to the disease.
Overview:	Provide hope that immunotherapy would have therapeutic potential to help those living with ALS.

Marie Gingras, PhD student
Where:	Currently working with Dr. François Berthod, a researcher and professor at Laval University
Project:	Developing a tissue-engineered model of the spinal cord in ALS research
Overview:	The objective for the project is to develop a three-dimensional model of a reconstructed spinal cord so that the interactions between different cell types and motor neurons can be studied.
Funding:	Holds a studentship from the Fonds de la Recherche en Santé, and funding for the project is provided by Muscular Dystrophy Association (U.S.)

Glen Hughes, Project Engineer
Where:	Institute of Biomedical Engineering in Fredericton
Project:	Currently working with Dr. Colleen O’Connell, a physiatrist at the Stan Cassidy Centre for Rehabilitation, to develop a proposal for a set of tools that would allow more efficient tracking of the progression of ALS.
Overview:	Eventually such tools could be used for evaluating the effects of different drugs in clinical trials, and to better monitor the diminishing strength of people who have ALS.

Teresa Sanelli, PhD Student
Where:	University of Western Ontario, under the supervision of Dr. Michael Strong
Project:	Working with primary motor neuron cells from transgenic mouse models of ALS that display ALS-like pathology (aggravates) in culture.
Overview:	Examining these processes could give indications as to how to more successfully treat the disease.

Dr. Sanjay Kalra
Where:	University of Alberta
Project:	Using magnetic resonance imaging (MRI) technology to learn more about what could be the cause of ALS and to find biomarkers. Biomarkers – also known as surrogate markers – are biologically-derived indicators that reflect the extent of the disease without having to physically examine tissue.
Overview:	Techniques based on MRI have the potential to improve our understanding of ALS and the way that clinical trials are done. University of Alberta Hospital Foundation and the MSI Foundation of Alberta

Dr. Shangxi Xiao
Where:	The Centre for Research in Neurodegenerative Disease at the University of Toronto
Project:	In his research, Xiao’s aim is to identify the cause of alternative splicing isoforms, proteins derived from the same gene but with distinct physical and sometimes biological properties; usually a splice variant in the disease.

Dr. Guy Rouleau Canada Research Chair, Genetics of the Nervous System
Where:	University of Montreal
Project: Funding:	Work in his lab has recently led to discovery that the disruption of the peripherin gene in humans accounts for a portion of ALS cases. The ALS Association (U.S.), the Muscular Dystrophy Association, Genome Canada, CIHR and National Institutes of Health

Dr. François Gros-Louis
Where:	Laval University
Project:	Currently screening candidate genes for a study on genetic causes of ALS. He is looking for other genes that may be responsible for the disease or that may affect onset and progression.

Dr. John Turnbull
Where:	McMaster University Hospital
Project:	Researching ways to implement viral delivery vehicles for RNAi (ribo nucleic acid interference). These techniques are being tested in transgenic mice, and also in cell cultures.
Overview:	Turnbull and his team are hopeful that RNAi will have a role in battling familial ALS and, later on, sporadic ALS.

Jason Wilson, PhD Student Recipient of the Brain Star Award (2005)
Where:	University of British Columbia with Dr. Chris Shaw
Project:	ALS-parkinsonism dementia complex
Overview:	Owing to the overlapping symptoms of several progressive neurological disorders, ALS-PDC may hold the key to unlocking important information about degenerative neurological disorders including ALS.

Dr. Christopher Shaw
Where:	University of British Columbia
Project:	Feeding washed cycad (ancient plant that creates molecules that are toxic) to mouse models to study ALS-parkinsonism dementia complex.
Overview:	With the ALS-PDC models, the disease can be observed in sequence allowing researchers to see what happens before symptoms begin to show.
Funding:	U.S. Army’s Defense Department, Natural Sciences and Engineering Research Council of Canada and the Scottish Rite Charitable Foundation of Canada, The ALS Association, ALS Society of Canada, National Institutes of Neurological Disease and Stroke, and Pacific Alzheimer's Research Foundation

Dr. Tim Doherty Canada Research Chair, Neuromuscular Function in Health, Aging and Disease
Where:	University of Western Ontario
Project:	Improving the ways of measuring disease progression in ALS and other disorders of the motor system.
Overview:	The ability to do so will be crucial to monitor the potential benefit of new treatments as they become available.
Funding:	Compumedics Limited in Australia, the CIHR, the Natural Sciences, Engineering Research Council of Canada (NSERC)

Dr. Neil Cashman Canada Research Chair, Neurodegeneration and Protein Misfolding Diseases
Where:	University of British Columbia Direct - The ALS Centre at G.F. Strong Rehabilitation Centre (B.C.) and the University of British Columbia

Overview:	Laboratory research focuses upon the cellular and molecular basis of neurodegenerative diseases.
Funding:	Temerty Family Trust and Amorfix Life Sciences

Dr. Robert Tanguay
Where: Project: Funding:	McGill University, Montreal Neurological Institute Over expression of small mitochondrial chaperones in a mouse model of ALS Joint ALS Society of Canada - The ALS Association grant (2007) - One year

David Gosselin, PhD Student
Where:	Université Laval
Project:	Increasing microglial expression of CCR2 and IGF-1 through genetic engineering of hematopoietic stem cells (HSC) for the treatment of ALS
Funding:	ALS Society of Canada – CIHR INHMA Doctoral Research Award (2007) - Three years

Dr. Xiaoyang Shan, MD, PhD Student
Where: Project: Funding:	Simon Fraser University The role of O-glycosylation in a mouse model of ALS ALS Society of Canada – CIHR INHMA Doctoral Research Award (2007) - Three years

Dr. Sherif Elbasiouny
Where:	Northwestern University
Project: Funding:	Ionic mechanisms underlying motoneuron degeneration in ALS Tim E. Noёl Fellowship in ALS Research (2007) - Three years

ALS Research News - The latest ALS als research articles.

1. Abnormal SOD1 folding is unique to familial ALS, researchers say

Mutations in the gene encoding superoxide dismutase 1 (SOD1) cause up to 20 per cent of inherited ALS cases (familial ALS). They’ve even been observed in a small fraction of sporadic cases. It has bee ◊
"Abnormal SOD1 folding is unique to familial ALS, researchers say"

2. Swimming-based exercise preserves motor neuron function

Whether exercise predisposes to or protects against amyotrophic lateral sclerosis is a topic of ongoing debate. ◊
"Swimming-based exercise preserves motor neuron function"

3. Synapse formation can be triggered artificially

The formation of synapses – the junctions through which neurotransmitters relay chemical signals allowing neurons to communicate information – typically requires contact between presynaptic and postsy ◊
"Synapse formation can be triggered artificially"

4. Phase 3 talampanel trial begins

Talampanel, a drug currently not approved in Canada or the United States, is undergoing an international Phase 3 trial involving 500 patients with ALS in centres across Europe, Canada and the US. In a ◊
"Phase 3 talampanel trial begins"

5. Genetic contributors to motor neuron diseases

The recent discovery of a number of genes known to cause motor neuron diseases such as ALS has provided new insights into the mechanisms underlying motor neuron degeneration, according to a recent art ◊
"Genetic contributors to motor neuron diseases"

6. Defining survival as an outcome measure for clinical trials in ALS

Clinical trials in ALS often use patient survival as an outcome measure to examine whether a treatment prolongs life. But variables affecting survival rates – such as emergency tracheostomy – are dif ◊
"Defining survival as an outcome measure for clinical trials in ALS"

7. Encouraging trial results for KNS-760704

At the 20th Internal Symposium on ALS/MND, researchers presented the results of a Phase 2 clinical trial testing the safety and tolerability of a drug called KNS-760704 for use by ALS patients. KNS-7 ◊
"Encouraging trial results for KNS-760704 "

8. Molecule discovered that might help ALS Patients

University of Texas researchers identified a molecule that reduces symptoms and prolongs life in mice with a type of ALS, according to a study in the Dec. 11, 2009, issue of Science. Lead author Eric ◊
"Molecule discovered that might help ALS Patients"