Sheila Essey Award for ALS Research
Sheila Essey Award for ALS Research

Sheila Essey Award Recipients

Winners of the prestigious Sheila Essey Award have made significant contributions to all avenues of ALS research. The award is made possible through the generosity of the Essey Family Fund in memory of Sheila Essey, who battled ALS for 10 years and died from the disease in 2004.  

2023   

Virginia M.Y. Lee, Ph.D., University of Pennsylvania  

  • Established how the buildup of misfolded protein in the brain contributes to the development of ALS and other neurodegenerative diseases  
  • Discovered the link between the TDP-43 protein and the development of frontotemporal dementia (FTD) and ALS 
  • Demonstrated that cell-to-cell transmission of pathological TDP-43 causes ALS to progress, opening up new avenues of research to identify targets for drug discovery  
  • Has been dedicated to mentoring younger colleagues, which will continue to impact the field for generations to come 

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2022  

Matthew Kiernan, MBBS, Ph.D., DSc, FRACP, FAHMS, Brain and Mind Centre, University of Sydney, Australia  

  • Played a pivotal role in improving care and driving ALS research worldwide through his stewardship of the Pan-Asian Consortium for the Treatment of ALS 
  • Established consensus guidelines for ALS that have helped simplified diagnosis and improve clinical trial enrollment 
  • Developed important techniques that enable study of disruption of brain networking in ALS 
  • Established new disease biomarkers and mechanisms of disease progression  

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2021  

Jan Veldink, M.D., Ph.D., University Medical Center Utrecht, The Netherlands  

  • Established a research line on ALS genetics, epidemiology and transcriptomics  
  • Established an international biobanking register and patient database specifically for ALS, which is essential to be able to carry out Project MinE   

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2020   

Guy Rouleau, M.D., Ph.D., McGill University, Canada  

  • Identified the super oxide dismutase (SOD1) gene  
  • Identified TARDBP as an ALS-linked gene – TDP-43, the protein made from this gene, has emerged as possibly the most important protein in sporadic ALS  
  • Identified FUS, providing a key RNA-binding protein alternative to TDP-43  
  • Identified the role of the hGle1 gene in ALS – the first time the nuclear pore was implicated in ALS  

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2019   

Aaron D. Gitler, Ph.D., Stanford University.   

  • Found a gene in yeast cells that could protect those cells from clumping TDP-43 proteins, which is thought to play a major role in ALS   
  • Developed a way to inhibit expression of C9orf72 transcripts with the potential of leading to therapeutic outcomes   
  • Found that mutations in the ataxin-2 gene were significantly associated with risk of ALS and that lowering levels of ataxin-2 in mice with antisense therapy can help mice with TDP-43 connected ALS live longer   

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2018   

Timothy Miller, M.D., Ph.D., Washington School of Medicine in St. Louis  

  • Instrumental in bringing antisense technology to clinical trials for people living with ALS   
  • Co-investigator for the SOD1 antisense clinical trials, along with developing relevant SOD1 biomarkers measured in cerebral spinal fluid and plasma   
  • Conducted important natural history studies for C9orf72 families, which were critical to inform C9orf72 antisense clinical trials   
  • Developed a biomarker important for tracking TDP-43, a protein found in almost all cases of ALS   
  • Identified that down regulation of microRNA (miR-155) in ALS mice could translate into a potential therapeutic target   

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2017   

John Ravits, M.D., University of California San Diego  

  • Revealed the pattern of ALS disease spread through the spinal cord and brain, which led to the seminal observation that disease spreading is consistent with transfer from cell to cell within the nervous system  
  • Helped pioneer antisense oligonucleotide therapy for the most common genetic form of ALS, C9orf72  
  • Served as principal investigator on many ALS clinical trials and as the director of the ALS Clinic at the University of California San Diego Health System, an ALS Association Certified Center of Excellence  

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2016   

Ammar Al-Chalabi, Ph.D., FRCP, DipStat, King’s College, London and King’s MND Care and Research Center  

  • Helped identify many known genetic causes of ALS, including C9orf72  
  • Developed the ALSoD database, an important tool for researchers funded by The ALS Association, MNDA UK and Therapy Alliance  
  • Made significant contributions to understanding disease staging   

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2015   

Robert Bowser, Ph.D., Barrow Neurological Institute  

  • Identified ALS biomarkers in the blood and cerebrospinal fluid   
  • Co-founded Knopp Biosciences and Iron Horse Diagnostics  
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2015   

Adriano Chio, M.D., F.A.A.N., University of Turin, Italy  

  • Established the Piedmont ALS registry to document genetic and environmental risks, progression and survival rates of people living with ALS  
  • First to describe the increased risk of ALS among Italian soccer players and the potential increased risk attributed to physical activity and traumas  
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2014   

Jeremy Shefner, M.D., Ph.D., Barrow Neurological Institute  

  • Developed an ALS biomarker to track ALS disease progression, called motor unit number estimate (MUNE)   
  • Co-founded the Northeast ALS Clinical Trials Consortium (NEALS) along with Merit Cudkowicz, M.D., to coordinate ALS clinical trial centers in northeastern U.S.  
  • Served as principal investigator of numerous clinical trials  
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2013   

Rosa Rademakers, Ph.D., Mayo Clinic, Jacksonville, FL  

  • Co-discovered the C9orf72 mutation, the most common genetic cause in ALS, from her study of a multigenerational family in North America  
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 2013 

Bryan Traynor, M.D., Ph.D., Laboratory of Neurogenetics, National Institute of Aging   

  • Co-discovered the C9orf72 mutation, the most common genetic cause in ALS, from his study of a Finnish population  
  • Discovered mutations in the VCP and MATR3 genes as causes of familial ALS  
  • Conducted the first genome-wide association study of ALS   
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2012   

Christopher Shaw, MBChB, M.D., FRACP, King’s College, London, UK  

  • Discovered TARDBP and FUS mutations as causes of familial ALS  
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2011   

Leonard van den Berg, M.D., Ph.D., University Medical Center, Utrecht, The Netherlands  

  • Founded the first integrated Netherlands ALS Center   
  • Completed population-based studies showing smoking to be a risk factor for ALS and that moderate alcohol consumption might be protective  
  • Initiated Project MiNE  
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2010   

Clive Svendsen, Ph.D., Cedars-Sinai Regenerative Medicine Institute, Cedars-Sinai Medical Center

  • Harnessed induced pluripotent stem cell (iPSC) technology using adult skin cells from people living with ALS and reprogrammed them to generate neuronal cell types, including motor neurons  
  • Helped develop surgical techniques used in stem cell trials   
  • Generated good manufacturing procedures and a clinical grade bank of human cells producing GDNF (growth factor)   
  • Established the iPSC core at Cedars-Sinai   
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2009   

Orla Hardiman, Ph.D., Trinity College Institute of Neuroscience, Dublin, Ireland  

  • Developed the longest running population-based ALS registry in the world  
  • Published a study showing that the frequency of ALS is not uniform around the world   
  • Linked a series of novel mutations in the gene angiogenin to some cases of ALS  
  • Showed that cognitive impairment in ALS occurs in about 40% of people and occurs early in the disease  
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2009   

Merit Cudkowicz, M.D., Harvard Medical School and Massachusetts General Hospital MDA ALS Clinic   

  • Co-founder and co-director of the NEALS Clinical Trials Network   
  • Investigator in numerous ALS clinical trials   
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2008   

Wim Robberecht, M.D., University Hospital Gasthuisberg, University of Leuven, Belgium  

  • Discovered disease-modifying genetic factors, such as the involvement of heat shock proteins and vascular endothelial growth factor (VEGF) in ALS, using genetic screening of zebrafish and fly models   
  • Established the role of excitotoxicity and calcium metabolism in motor neuron death in ALS  
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2007   

Christopher Henderson, Ph.D., Columbia University Medical Center, Motor Neuron Center and Columbia Stem Cell Initiative   

  • Pioneered the discovery of helper molecules called trophic factors that support the growth and health of motor neurons  
  • Helped describe the role of nitric oxide in programmed cell death  
  • Co-founded Trophos, which had a lead molecule successful in spinal muscle atrophy trial   
  • Served as vice president of neurology at Biogen  
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2006   

Peter Carmeliet, M.D., Ph.D., Center of Transgene Technology and Gene Therapy, University of Leuven, Belgium  

  • Discovered the role of VEGF in motor neuron disease  
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2005   

Michael Strong, M.D., FRCPC, Western University, Ontario, Canada   

  • Served as co-chair of the Canadian ALS Research Consortium   
  • Contributed to an understanding of the mechanisms by which intracellular deposits of protein (composed primarily of neurofilament) are formed and how these aggregates contribute to the disease process of ALS  
  • Demonstrated that cognitive changes in ALS are associated with alterations in the metabolism of tau protein  
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2004   

P. Nigel Leigh, M.B.B.S., Ph.D., F.R.C.P., FMedSCI, University of Sussex, UK  

  • Established a research program in Motor Neuron Disease at King’s College London – the King’s MND Care and Research Center was the first center in the UK funded by the MNDA and is a model for multi-disciplinary care worldwide.   
  • Made significant contributions to imaging techniques for ALS  
  • Contributed to the development of riluzole, the first FDA-approved drug to treat ALS   
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2003   

Stanley Appel, M.D., Institute for Academic Medicine, Houston Methodist and ALS/MDA Clinic at Methodist Neurological Institute   

  • First to develop an immune-mediated preclinical model of motor neuron disease and then document the presence of neuroinflammatory markers in both the preclinical model and human sporadic ALS  
  • Documented enhanced neuroinflammation and activated microglia prior to evidence of clinical disease and motor neuron cell death  
  • Demonstrated the presence of increased intracellular calcium and altered mitochondria in human sporadic ALS motor neurons to help explain the selective vulnerability of motor neurons in ALS  
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2002   

Serge Przedborski, M.D., Ph.D., Columbia University  

  • Pioneered the investigations of motor neuron death in the SOD1 model of ALS  
  • Demonstrated the importance of the cascade of deleterious events, such as oxidative stress, inflammation and programmed cell death as related to the health of motor neurons in the spinal cord in ALS  
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2001   

Dame Pamela Shaw, M.B.B.S., M.D., F.R.C.P., University of Sheffield, UK  

  • Increased understanding of the cellular pathways of motor neuron injury in the presence of SOD1 gene mutations  
  • Identified the different subtypes of motor neuron disease based on genetics and molecular pathology   
  • Established the role of non-invasive ventilation in improving quality of life and prolonging survival of people with motor neuron disease  
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2000   

Jean Pierre Julien, Ph.D., Université Laval, Quebec City, Canada   

  • Made significant contributions to our understanding of the role of neurofilaments in ALS as well as the pathogenesis of SOD1 mutations  
  • Developed several novel mouse models and used them to test ALS therapeutics   
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1999   

Don Cleveland, Ph.D., Ludwig Institute for Cancer Research, University of California San Diego 

  • Identified the protein tau  
  • Identified key steps that trigger disease and accelerate ALS progression  
  • Established the role of non-neuronal cells, including astrocytes and oligodendrocytes, in ALS disease and promoted the idea that disease is not cell autonomous   
  • Pioneered the use of antisense oligonucleotides as a therapy for human neurodegenerative disease 
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1998   

Ted Munsat, M.D., Tufts University School of Medicine  

  • Served as president of the American Academy of Neurology, 1989-1991; chair of the Continuing Educational Committee of the AAN; and founding director of AAN’s premier continuing medical education journal Continuum: Lifelong Learning in Neurology   
  • Chaired the World Federation of Neurology (WFN) ALS Research Group that published important international diagnostic guidelines  
  • Pioneered many outcome measures that are still in use in clinical trials today  
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1998   

Lewis Rowland, M.D., Columbia University College of Physicians and Surgeons  

  • Chaired the Department of Neurology at Columbia for 25 years and also served as chair at the University of Pennsylvania.   
  • Co-founded the Eleanor and Lou Gehrig MDA/ALS Center   
  • Served as president of the American Academy of Neurology, the American Neurological Association and the Association of Professors of Neurology   
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1997   

Jeffrey Rothstein, M.D., Ph.D., Johns Hopkins School of Medicine  

  • Contributed to the development of the first FDA-approved drug for ALS, riluzole  
  • Provided the first evidence that glial dysfunction could contribute to and accelerate neurodegenerative disease  
  • Responsible for many ALS therapeutic candidates, including small molecules, gene therapies and cellular-based therapeutics     
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1996   

Robert Brown, Jr., M.D., Ph.D., University of Massachusetts Medical School  

  • Discovered the first ALS gene, SOD1, in 1993, which led to the creation of the first mouse model of ALS in 1994  
  • Played a central role in the discovery of other mutations or genetic variants in several ALS-related genes including ALS2, DCTN1, KIFAP3 and FUS/TLS  
  • Reported evidence suggesting that the SOD1 gene also played a role in sporadic ALS   
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1996   

Teepu Siddique, M.D., Northwestern University Feinberg School of Medicine  

  • Initiated the molecular genetic approach to ALS in 1984, which lead to the successful identification of many genetic causes for ALS, including SOD1, ALSIN, PON, FUS, TDP-43, SQSTM1 and UBQLN2   
  • Identified a common pathology for all ALS involving UBQLN2 and SQSTM1  
  • Engineered several models for ALS, including the first genetic model for neurodegeneration, SOD1  
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Sheila Essey Award for ALS Research