£1.5m available for research on disorders affecting motor neurones including post-polio syndrome

August 2014

Three researchers

£1.5m available for research on disorders affecting motor neurones including post-polio syndrome

 

Diseases and conditions affecting motor neurones can be devastating.

Motor neurones are the cells that control essential muscle activity such as speaking, walking, swallowing and breathing. If motor neurones die, the nerve messages they carry gradually stop reaching the muscles leading to weakness, wasting and often death. For people diagnosed with a disorder affecting their motor neurones, and for their families, the outlook can look grim. There is no cure.

Our donors want to tackle this and so the MRF is making £1.5m available to the very best UK researchers to undertake research to understand better these rare disorders and, in doing so, try to improve the outlook for future generations. There are a range of disorders affecting motor neurones:

Motor neurone diseases (MND) are a group of progressive neurological disorders that destroy motor neurones and lock people into failing bodies.  They are severely life-shortening and kill five people each day in the UK, half within 14 months of diagnosis, and 100,000 people worldwide every year. Only 10% of cases are inherited, the other 90% arising sporadically.

Spinal Muscular Atrophy (SMA) is a neuromuscular disease that is inherited and also affects the motor neurones responsible for muscle function.  SMA is the leading genetic killer of infants and toddlers, with approximately 95% of the most severely diagnosed cases resulting in death by the age of 18 months. 1 in 40 people is an SMA carrier in the UK and 1 in every 6,000 births is affected by SMA.

Kennedy’s disease is an inherited disease caused by a genetic defect on the X (female) chromosome. Since males have only one X chromosome, they are most severely affected by the disorder which results in motor neurone death and muscle wasting. Females may have the mutation, but as they have two X chromosomes they do not show the effects of the mutation. For female carriers, however, 50% of their sons may get the disease, and 50% of their daughters may become carriers. Kennedy’s disease is estimated to occur in 1 in 40,000 individuals worldwide.

Post-polio syndrome (PPS) is a condition that can strike between 25 and 50% of individuals who have recovered from paralytic polio decades after their recovery during childhood, leading to further weakness and disability.  Polio is an acute viral disease that can destroy motor neurons in those most severely infected leading to muscle weakness and paralysis.  After acute polio, the surviving motor neurons expand the amount of muscle that each controls.  PPS is thought to occur when the surviving motor neurons are lost in the aging process or through injury or illness. PPS is a slow progressive disease and there is no cure.

 

A number of generous MRF donors have left money in their Wills to help address these problems and create a brighter future the individuals and families of those affected by these disorders. We will be able to make a difference thanks to the support of the late Irene Griffiths, in memory of her parents Ena and Harold Griffiths, Edna Brierley, Margaret Elt, Evelyn Schuster and John Stephenson.

If you would like to help UK scientists make a difference then please donate now.

If you are a UK scientist with the skills, experience and passion to increase understanding of these devastating diseases then we would like to hear from you.