Motor neuron disease (MND) is a progressive neurodegenerative condition that attacks motor neurons — specialised nerve cells in the brain and spinal cord — causing loss of signals from the brain to muscles and eventually leading to paralysis.
The disease affects 300 Kiwis at any one time and the charity funding the research project, the Motor Neuron Disease Association, says it affects 5,000 people in Britain.
Dr Miles said researchers adopting stem cell technology could now turn samples of human skin into a replica of a motor neuron for laboratory study. "We can't go in and grab people's motor neurons out of a spine. But this way we have an actual human cell we can grow in a dish and study and test."
The function of motor neurons from healthy people could be compared with those from patients suffering from different forms of MND.
A report on the joint project between St Andrews and University of Edinburgh scientists was published this week by the journal Nature Communications.
The work has so far shown that before any signs of damage, motor neurons affected by MND lose the ability to generate the electrical signals to the muscles due to changes in proteins called ion channels.
"Our work highlights ion channels as potential targets for future therapies," said Dr Miles.
"It also demonstrates that studying the function of stem cell-derived motor neurons could be important for the development and testing of new drugs to treat and eventually cure the disease."
Motor neuron disease
• Attacks motor neurons — nerve cells in the brain and spinal cord that control movement.
• People can still think and feel but become unable to move, talk and eventually breathe.
• 5,000 people in Britain have it at any one time.
• 5 die of the disease every day in Britain.
• 300 people in NZ suffer from it at any one time.
• Stem cell-derived motor neurons allow closer study and tests for a cure.