A cure for motor neurone disease – which killed Professor Stephen Hawking – has moved one step closer, scientists believe.
The cruel condition causes signals from motor neurone nerve cells in the brain and spinal cord to fail.
Motor neurones control crucial muscle activity and if they are damaged and break down some patients eventually find it impossible to walk or even speak.
Now researchers have found that another type of brain cell – thought to be harmless – could play a role in the disease, which is also known as ALS (amyotrophic lateral sclerosis).
There is currently no known cure for motor neurone disease. The cruel condition killed Professor Stephen Hawking, pictured
Glial cells under a microscope are now said to be ‘key players’ in motor neurone disease
Tests of cells from skin samples of patients with MND, also known as ALS, showed glial cells can damage motor neurones.
University of St Andrews researchers said glial cells normally support neurones in the brain and spinal cord.
They tested different combinations of glial cells and motor neurones grown together in the lab to test their discovery further.
They found glial cells from MND patients caused motor neurones of healthy people to stop producing the electrical signals needed to control muscles.
Pete Frates, the former college baseball star whose battle with ALS inspired the Ice Bucket Challenge, has passed away at the age of 34, his family announced Monday
In 2014 he launched the ALS Bucket Challenge, a viral sensation in which people dumped buckets of ice on themselves, donated to ALS research, and nominated friends to do the same. The challenge raised a whopping $200million worldwide. Frates pictured above participating in the challenge surrounded by his family
WHAT IS AMYOTROPHIC LATERAL SCLEROSIS?
ALS is an acronym for amyotrophic lateral sclerosis.
It is also referred to as motor neurone disease, or Lou Gehrig’s Disease after the US baseball player when he was diagnosed in 1939 at just 36 years old.
The disease is a rare condition that progressively damages parts of the nervous system.
It occurs when specialist nerve cells in the brain and spinal cord called motor neurones stop working properly – known as neurodegeneration.
Life expectancy for about half of those with the condition is three years from the start of symptoms.
However, some people may live for up to 10 years, and in rarer circumstances even longer.
The condition can affect adults of all ages, including teenagers, although this is extremely rare.
It’s usually diagnosed in people over 40, but most people with the condition first develop symptoms in their 60s. It affects slightly more men than women.
There’s currently no cure for motor neurone disease.
Treatment aims to make the person feel comfortable and have the best quality of life possible
It also tries to compensate for the progressive loss of bodily functions such as mobility, communication, swallowing and breathing.
Professor Gareth Miles, who helped lead the joint project, said: ‘We are very excited by these new findings.’
He added they ‘clearly point the finger at glial cells as key players in this devastating disease’.
Professor Miles said: ‘Interestingly, the negative influence of glial cells seems to prevent motor neurones from fulfilling their normal roles, even before the motor neurones show signs of dying.
‘We hope that this new information highlights targets for the development of much-needed treatments and ultimately a cure for MND.’
Almost 15,000 Americans have ALS. The NHS says there are about 5,000 people in the UK living with a motor neurone disease.
The research, which involved a team from the University of Edinburgh, was funded by the Motor Neurone Disease Association, MND Scotland and the Medical Research Council and Dementia Research Institute, Edinburgh.
The results were published in the scientific journal Glia.
Dr Brian Dickie, director of research at the Motor Neurone Disease Association, said: ‘The human brain has at least as many support cells, called glial cells, as it does neurons.
‘Whilst glial cells usually play a nurturing and supportive role, research has shown that they may accidentally play a role in a number of neurological diseases including motor neurone disease by affecting the speed at which the disease spreads though the central nervous system – a little like the way the strength of the wind can inadvertently fan the flames of the forest fire.
‘The researchers have shown that a particular type of glial cell known as an astrocyte appears to impede the action of motor neurons, which then become progressively dysfunctional.
‘Further research will be needed to show that the effects seen in cells grown in the lab are also happening in people with motor neurone disease.
‘If so, then it could open up promising new approaches aimed at slowing the progression of the disease.’
ALS is a neurodegenerative disease that effects the muscles used for walking and talking. Patients have a life expectancy of about three to 10 years.
But the world-renowned physicist Professor Hawking lived with the disease for more than five decades before his death in 2018.
The cause of ALS is not known, but scientific studies suggests that both genetics and environment play a role in the development.
The discovery comes after Pete Frates, the former college baseball star whose battle with ALS inspired the Ice Bucket Challenge, died last week at the age of 34.
Frates, of Massachusetts, was a symbol of hope for people with ALS and helped raised awareness and over $200million for research on the neurodegenerative disease that weakens muscles and impacts movement.