Fridays with Phil

Earlier this month, over 700 people walked the outskirts of Lake Macquarie to raise awareness of MND and help raise finances to find a cure.  The cure may be closer than we realise with the below article published Friday in the Proceedings of the National Academy of Sciences USA.

Your continued prayers that a cure be found sooner rather than later for the 1,800 people suffering with this disease in Australia, and many more around the world, would be much appreciated.

We do not give up hope.
Phil

Friday, February 21, 2014

Culprit protein in spread of motor neuron disease discovered

A breakthrough study has revealed how the fatal neurodegenerative disorder motor neurone disease (MND) is transmitted between nerve cells, and suggests the spread of the disease could be halted.

“The agent of spread has been discovered,” says Dr Bradley Turner, of the Florey Institute of Neuroscience and Mental Health.

Motor neurone disease is the name given to a group of diseases in which the nerve cells (neurones) controlling the muscles that enable us to move, speak, breathe and swallow undergo degeneration and die.

Typically, MND starts in a finger or a toe and then spreads. Gradually, it is transmitted throughout the nervous system causing paralysis and death – usually within 27 months. MND affects around 300,000 people worldwide and two Australians die from the disease every day.

“By understanding how the disease spreads in the brain, we can develop new strategies to combat the progressive symptoms seen in MND,” Dr Turner says.

The research shows that a misfolded protein can spread throughout the nervous system. The culprit protein is known as SOD1. The misshapen SOD1 spreads inside a living cell, from one neurone to another, like an infection. Importantly, the study reveals that “wild-type” or normal SOD1 can misfold and transmit between cells, which has implications for the common sporadic form of MND.

Published today in the Proceedings of the National Academy of Sciences USA, the study also shows the spread can be neutralised using antibodies. Antibodies bind to regions of misshapen SOD1, and block its spread. If SOD1 misfolding is the common culprit in MND, as the study suggests, then the antibodies could arrest MND progression, the researchers say.

No human clinical trials have taken place but studies in mice have been successful in blocking the misfolded SOD1 using antibodies and slowing MND symptoms.

The research could also have implications for those studying other neurological disorders  including Alzheimer’s and Parkinson’s diseases where spread of misfolded proteins is implicated. These diseases may resemble the most common human form of prion disease, Creutzfeldt-Jakob disease (CJD).

This discovery is the culmination of several years of work by an international team involving Dr Turner at the Florey, Prof Andrew Hill at the Bio21 Institute, University of Melbourne, Dr Justin Yerbury at the University of Wollongong and Prof. Neil Cashman at the University of British Columbia, Vancouver,

For media enquiries: Amanda Place at the Florey 0411 204 526