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Professor Pamela Shaw, Professor of Neurology, is one of only 16 figures across the country to be given a Damehood in recognition of her extraordinary contribution to neurosciences including revolutionary research in pioneering treatments for Motor Neurone Disease (MND). She is one of a number of leading figures from the University to receive gongs in the honours list. Professor Shaw, also a consultant neurologist at Sheffield Teaching Hospitals Foundation Trust, is the director of the University’s Sheffield Institute for Translational Neurosciences (SITraN), an £18m research facility bringing together 150 international clinicians and scientists to fight crippling diseases such as MND.
She said her award had come as a “wonderful surprise” adding: “The honour and prestige associated with the DBE award shines a beacon on to the work being done in SITraN and the Royal Hallamshire Hospital, Sheffield. Our vision is to harness the tremendous advances in neuroscience and translate these into benefits for people afflicted with motor neurone disease and related neurodegenerative conditions. I hope that I will be able to deploy this beacon of recognition to help with our work and that 2014 will bring a significant positive breakthrough to benefit patients and families facing these devastating conditions.”
Congratulating Professor Shaw on her DBE, Professor Sir Keith Burnett added: “Professor Shaw continues to act as an inspiration to colleagues across the world who rightly hold the work of SiTraN in the very highest respect. The most important honour of all lies in the difference Professor Shaw and her colleagues make to the health and well-being of men, women and children suffering some of the most debilitating diseases we know. As a University we are proud of all Professor Shaw has achieved, and I am personally delighted.”
Tigar, a newly discovered protein in the world of Parkinson's, could be the key to stopping the degenerative disease according to research from the University of Sheffield.
Scientists from the Sheffield Institute of Translational Neuroscience (SITraN) and the MRC Centre for Developmental and Biomedical Genetics (CDBG), both University of Sheffield, found that by blocking the protein, nerve cells usually lost in the progression of Parkinson's could be saved – potentially halting the spread of the condition.
The ground breaking research, published in Annals of Neurology, was conducted by Dr Oliver Bandmann and his team who looked at the cell death of dopamine-producing nerve cells in zebrafish with a mutation in a gene called PINK1. PINK1 is linked to a rare, inherited form of early-onset Parkinson’s in humans. The team discovered that Tigar became overactive in the PINK1 zebrafish suggesting that it may be involved in causing the death of nerve cells, triggering the start of Parkinson’s. By blocking the activity of Tigar, the researchers were able to save the dopamine-producing nerve cells. It is these precious cells which are also lost in people with Parkinson’s. So, if blocking Tigar activity has the same effect in people it could have the potential to stop the spread of Parkinson’s.
Comparing rapidly progressing with slowly progressing MND mouse models revealed key differences in major molecular pathways and the way the protective systems of the body responded.
The research published in the scientific journal Brain will provide MND researchers with valuable clues about mechanisms and treatment strategies that lead to slowing down the progression of disabling symptoms in MND and was conducted by Professor Pamelas Shaw's team in SITraN in collaboration with Dr Caterina Bendotti and her group at the Mario Negri Institute for Pharmacological Research in Milan, Italy, and was funded by the Motor Neurone Disease Association.
The teams from Sheffield and Milan looked at the factors which might explain the differences why the progression of MND following onset of symptoms varies in speed and severity. In the case of the model with rapidly progressing MND the motor neurones showed reduced functioning of the cellular systems for energy production, disposal of waste proteins and neuroprotection. Motor neurones from the model with more slowly progressing MND showed an increase in protective inflammation and immune responses and increased function of the mechanisms that protect motor neurones from damage.
The evidence found for key changes at the point of onset of the disease, before muscle weakness was observed, will give researchers new insights as to how the debilitating disease affects individual patients and how new treatments could be targeted. The MND mouse models investigated carry an alteration in the SOD1 gene, which is a known cause of MND in humans. The scientists used a scientific technique known as gene expression profiling to identify factors within motor neurones that control vulnerability or resistance to MND in order to shed light on the factors important for the speed of motor neurone injury in human patients.
On the afternoon of Tuesday 17th September 2013 SITraN welcomed over a 100 visitors during the Institute’s second annual public open day. Visitors included patients, carers, families as well as members of the public.
SITraN researchers were privileged to be given the opportunity to talk to patients and the public about the research they carry out into neurodegenerative diseases including Motor Neurone Disease (MND), Spinal Muscular Atrophy (SMA), Parkinson’s Disease (PD), Alzheimer’s Disease (AD) and Dementia. The event began with a warm welcome from Professor Pamela Shaw, Director of SITraN and an update on the Institute’s pioneering MND research, followed by talks given by Dr Oliver Bandmann on Sheffield’s Parkinson’s Disease research and Dr Chris McDermott on how Sheffield is championing the development assistive technologies to improve the quality of life for patients with neurodegenerative disease such as the “Sheffield Support Snood” for patients suffering from neck muscle weakness.
On the 16th of September, Professor Pamela Shaw (Director, Sheffield Institute for Translational Neuroscience and Professor of Neurology) hosted a delegation from King Fahad Medical City (KFMC), Riyadh, Saudi Arabia following a previous successful visit to the medical city in Riyadh in May 2012. KFMC is one of the biggest specialised hospitals in the Kingdom of Saudi Arabia, where it has a Clinical department concerned with tertiary level patient care, which offers teaching for health care students, training of medical students and residents in various medical fields, in addition to research.
The overall aim of both visits between the two institutes is to establish a collaboration from which both countries will significantly benefit. KFMC is in the process of establishing a translational neuroscience research centre as part of the National Neuroscience Institute (NNI) a regional referral centre for neurological disorders. SITraN is a world recognised centre of excellence that combines clinicians, scientists, technicians as well as students under one roof that are all dedicated to the study of neurodegenerative diseases and developing therapies that could offer better treatment options and quality of life for patients. A collaboration with Saudi Arabia offers a number of interesting opportunities to explore new clinical studies due to the high number of some clinical disorders in Saudi Arabia such as Spinal Muscular Atrophy (SMA) in children. Such studies have never been done in the Middle Eastern population and might offer new insights into specific neurological disorders adding to studies based on Caucasian populations. Linkage studies could be easily performed for the purpose of genetic hunting of certain neurological diseases in Saudi because of the culture of this country where relative marriage is a well-known tradition.