Working together to fight devastating neurodegenerative diseases

Dr Emily Goodall

Giving hope for patients with a cruel disease

Finding a cure for debilitating degenerative diseases, such as Motor Neurone Disease (MND), is moving ever closer thanks to world-class research conducted at the Sheffield Institute for Translational Neuroscience (SITraN) which has the potential to transform millions of lives across the world.

Groundbreaking researchSITraN – at the University of Sheffield – was officially unveiled by Her Majesty The Queen and His Royal Highness The Duke of Edinburgh in November 2010 with the vision to overcome the devastating effects of MND, which is an incurable disease, on patients and their families.

Two years on SITraN has grown immensely and developed into a leading global facility which is at the forefront of research, expertise and pioneering new treatments for MND and related neurological diseases such as Parkinson's, Spinal Muscular Atrophy (SMA) and Alzheimer's Disease- bringing new hope to patients and families across the country.

A world-class facility which is leading the way

The £18 million centre brings together state-of-the-art laboratories and equipment including a clinical database of over 1500 patients and the largest resource of human brain-bank material in the world.

Professor Pamela Shaw, Head of the Department of Neuroscience at the University of Sheffield and Director of SITraN said: "The vision behind the creation of SITraN was to establish a world class research institute where teams of clinicians and scientists could be brought together under one roof to focus their combined skills on solving the problem of MND and related neurodegenerative conditions.

"We aim to harness some of the exciting developments in neuroscience and translate these new approaches into benefits for our patients in the clinic. Many wonderful patrons, supporters, patients and family members got behind this vision and helped make it happen."

Professor Shaw added: "Outstanding benefits have already materialised. SITraN is acting as a magnet attracting top quality researchers from around the world to come to Sheffield, as well as research funding, philanthropic donations and pharmaceutical industry partners to support our work.

MND: the facts

  • MND is a progressive disease that attacks motor neurones, or nerves, in the brain and spinal cord.
  • MND leaves people unable to walk, talk or feed themselves, but the intellect and the senses usually remain unaffected.
  • The disease affects nearly twice as many men as women.
  • MND is typically fatal within 2–5 years of symptom onset.

"Several of our research programmes have already made a difference to life expectancy and quality of life for people afflicted by MND and more exciting and innovative programmes are in the pipeline. SITraN staff are immensely grateful to all of our supporters and to the MND sufferers who willingly give their time, energy and enthusiasm to help our research projects."

More effective therapies are desperately needed

Over 6,000 people in the UK suffer from MND. It is an incurable disease which destroys the cells that control movement causing progressive disability. Sufferers may lose their ability to walk, talk, eat and breathe. At present, treatment options for the protection of motor neurones have only a modest effect and more effective therapies are urgently needed.

SITraNOver the past 24 months discoveries made by the SITraN team have attracted more than £7.3 million of new research grant funding for MND and SMA, a childhood form of MND.

Expert researchers are successfully using a pioneering technique called gene expression profiling, which measures the activity of thousands of genes at once to establish a global picture of cellular function, to uncover several pathways important in motor neurone injury. Exploring these pathways will pave the way for the development of new therapies for the protection of motor neurones.

As well as developing life-changing therapies, SITraN is helping to inspire researchers of the future by enhancing the flow of young scientists and neurologists into MND research through the launch of new MSc courses in Translational Neuroscience and Clinical Neurology.

The motivation behind a inspirational researcher

Dr Emily Goodall, 31, was motivated to become a MND researcher after losing her father to the disease in 1998. She said: "In 1994 my father was told he had a progressive disability that meant he could never work again – he was just 50 years old.

"It was a terrible shock to us all, I was 13, my sister Helen was 11, and we didn't really understand how serious it was. Dad had to give up the job he loved as a gardener and the house we lived in as it was tied to his work."

Her Majesty The Queen unveils SITraNEmily's father was finally diagnosed with MND in 1995, by which time her family had moved into a new home with just a muddy patch of earth for a garden. Her dad relished the challenge and although he could no longer do it all himself he still took great pleasure in turning the unpromising terrain into a lovely garden.

"In November 1998, at the age of 54, dad lost his battle with MND. He died peacefully at home leaving us with wonderful memories, a beautiful garden and a shared passion for nature and biology that has influenced us all," said Emily.

"The year after dad's death I took my A level exams and got a place at Cardiff University to study genetics. Losing my father to MND motivated me to pursue research into MND and I feel like SITraN is the best place to do this. I remember seeing the blue prints for the SITraN building in my first meeting with Professor Pamela Shaw back in 2008 and it has been incredible to see everything come together.

"I hope my project will play a role in improving the diagnostic process for future families affected by MND, preventing the months of uncertainty we faced and helping them to get the support they need more quickly. I feel privileged to be part of the SITraN team, working in MND research has given me immense optimism that a cure can be found and I hope the work we do has a role to play in a future without this disease."

Additional Information

Fast Facts

Motor Neurone Disease

• MND is a progressive disease that attacks motor neurones, or nerves, in the brain and spinal cord.
• It can affect any adult at any age – although most people diagnosed with the disease are over 40.
• The disease affects nearly twice as many men as women.
• Approximately two in every 100,000 people are diagnosed with the condition.
• MND leaves people unable to walk, talk or feed themselves, but the intellect and the senses usually remain unaffected.
• People with MND can still think and feel, but their muscles refuse to work.
• MND is typically fatal within 2–5 years of symptom onset.
For more information visit the Motor Neurone Disease Association MNDA

Parkinson's Disease
• One person in every 500 has Parkinson's.
• It affects about 127,000 people in the UK.
• Most people who get Parkinson's are aged 50 or over but younger people can get it too. One in 20 is under the age of 40.
• People with Parkinson's don't have enough of a chemical called dopamine because some nerve cells in their brain have died.
• Without dopamine people can find that their movements become slower so it takes longer to do things.
• There's currently no cure for Parkinson's and we don't yet know why people get the condition.
For more information about Parkinson's Disease visit Parkinson's

Alzheimer's Disease
• Alzheimer's disease is the most common cause of dementia, affecting around 496,000 people in the UK.
• Dementia affects one in 14 people over the age of 65 and one in six over the age of 80.
• More than 17,000 people under the age of 65 are living with dementia.
• Symptoms of Alzheimer's disease can include loss of memory, mood changes and problems with communication and reasoning.
• Alzheimer's is a progressive disease which means over time more parts of the brain are damaged and the symptoms become more serve.
To find out more about Alzheimer's Disease visit Alzheimer's

SITraN
For more information about the Sheffield Institute for Translational Neuroscience visit SITraN

The University of Sheffield
With nearly 25,000 students from 125 countries, the University of Sheffield is one of the UK’s leading and largest universities. A member of the Russell Group, it has a reputation for world-class teaching and research excellence across a wide range of disciplines. The University of Sheffield was named University of the Year in the Times Higher Education Awards 2011 for its exceptional performance in research, teaching, access and business performance. In addition, the University has won four Queen’s Anniversary Prizes (1998, 2000, 2002, and 2007).

These prestigious awards recognise outstanding contributions by universities and colleges to the United Kingdom’s intellectual, economic, cultural and social life. Sheffield also boasts five Nobel Prize winners among former staff and students and many of its alumni have gone on to hold positions of great responsibility and influence around the world. The University’s research partners and clients include Boeing, Rolls-Royce, Unilever, Boots, AstraZeneca, GSK, ICI, Slazenger, and many more household names, as well as UK and overseas government agencies and charitable foundations.

The University has well-established partnerships with a number of universities and major corporations, both in the UK and abroad. Its partnership with Leeds and York Universities in the White Rose Consortium has a combined research power greater than that of either Oxford or Cambridge.

Contact

For further information please contact:

Amy Pullan
Media Relations Officer
The University of Sheffield
0114 222 9859
a.l.pullan@sheffield.ac.uk