Lecture Details

Motor Neuron Disease
Professor Dame Pamela Shaw

Vice President and Head of the Faculty of Medicine, Dentistry and Health, University of Sheffield

Professor of Neurology, University of Sheffield

Director, Sheffield Institute for Translational Neuroscience (SITraN)

Director, NIHR Biomedical Research Centre Translational Neuroscience for Chronic Neurological Disorders

Director, Sheffield Care and Research Centre for Motor Neuron Disorders

Consultant Neurologist, Sheffield Teaching Hospitals NHS Foundation Trust

Professor Shaw is a Clinician Scientist in Neurology, an NIHR Senior Investigator and formerly a Wellcome Senior Clinical Fellow. Supported by long-term programme funding from the Wellcome Trust, she has since 1991 led a major multidisciplinary programme of research investigating genetic, molecular and neurochemical factors underlying neurodegenerative disorders of the human motor system. Her team use robust cellular and in vivo models of neurodegeneration and correlate key findings with disease related changes in the human nervous system. Significant research achievements include:

  1. Identification of cell specific features of motor neurons which underlie susceptibility to neurodegeneration.
  2. Understanding the cellular pathways of motor neuron injury in the presence of mutant SOD1 using a combination of cell biology, proteomics and gene expression profiling.
  3. Identifying the different subtypes of motor neuron disease (MND) based on genetics and molecular pathology.
  4. Development of new small molecule and gene therapy candidate treatments for patients using staged screening programmes in cellular and other experimental model systems.
  5. Promoting the introduction of one neuroprotective drug for MND into clinical practice – riluzole.
  6. Establishing the role of non-invasive ventilation (NIV) in improving the quality of life and prolonging survival of MND patients.

Professor Shaw’s research is currently funded by the Medical Research Council, NIHR, the Motor Neurone Disease Association, the European Union and biotechnology & pharmaceutical industry partners. Since 1983 she has generated more than £56m in research income.