Scientists from the University of Sheffield in the UK have identified new messenger molecules shuttled between cells which could help to protect the survival of neurones, potentially leading to new treatments for Motor Neurone Disease (MND). MND or Amyotrophic Lateral Sclerosis (ALS) is a neurological disorder in which the nerve cells -- neurones -- controlling the muscles that enable us to move, speak, breathe and swallow undergo degeneration and die.
Patients with ALS experience the loss of motor neurons and progressive paralysis. Following a long diagnostic journey, they may survive up to five years. Two ALS drugs have been approved by the US Food and Drug Administration (FDA), but they act only to slow the disease.
About 10 years ago, scientists found aggregates of a protein called TDP-43 in post-mortem neurons from ALS patients. This protein should have been in the nucleus of those neurons, but instead it was being flushed out, and building up in the cytoplasm.
The disorder affects 5,000 adults in the UK and there is currently no cure. The pioneering research has discovered the role of a small molecule which can regulate large signalling cascades and significantly improve the survival of neurones, something which will help pave the way to identify and develop new therapies for neuro-degenerative diseases.
Approximately 10 per cent of the MND cases are inherited. The remaining 90 per cent cases are caused by complex genetic and environmental interactions, which are currently not well understood.