Biological key to motor neurone disease identified
A breakthrough in motor neurone disease research has come about after scientists identified a protein that recycles damaged proteins in motor nerves.
Approximately 5,000 people have the disease in the UK, which can be fatal within three years, and scientists have so far been unable to put their finger on what causes it.
However, the identification of the ubiquilin 2 protein is a major breakthrough for researchers as they say it is pivotal in recycling damaged or misshapen proteins in motor nerves.
Neurons rely on the efficient recycling of proteins in the cells to help them operate properly. But when this system breaks down, the nerve cells are unable to fix or maintain themselves and become severely damaged as a result.
The find may also be able to help scientists progress their research into other neurodegenerative conditions such as Parkinson’s and Alzheimer’s, according to the researchers writing in an early online edition of the journal Nature.
Lead researcher Professor Teepu Siddique, from Northwestern University in Chicago, said: “This opens up a whole new field for finding an effective treatment for ALS.”
- Deng H, et al. Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Nature 2011; Advance online publication.