Toxic Proteins Responsible for Nerve Cell Damage

A new research found a specific genetic mutation damages nerve cells in two serious afflictions - frontotemporal dementia and motor neurone disease.

Researchers at the University College London and Max Planck Institute for Biology of Ageing stated that in rare cases patients might also suffer both these diseases.

Amyotrophic lateral sclerosis is a fatal motor neuron disease that rapidly weakens muscles. Frontotemporal dementia is the second most common cause of dementia in people under the age of 65. The disease comes with distressing symptoms such as changes in personality and behavior problems related to language and thinking. 

The faulty gene carries thousands of repeats of a genetic code. This repeated code results in extra molecules called RNA, as well as repeated fragments of protein..

Dr Adrian Isaacs of UCL's Institute of Neurology, senior author of the research, said in a press release, "These results suggest a key role for these toxic proteins in FTD and motor neurone disease, and the next step will be to understand whether drugs could be designed to target these proteins and stop the death of nerve cells. We believe these results mark an important advance in our understanding of these neurodegenerative diseases, and we are excited to follow up these findings.

Researchers had previously thought that the problem might be due to consequence of disruption of the gene by the inserted repeats. Another theory had that the repeats produce a different type of toxic RNA molecule. But, the current study shows that the repeats in the mutant gene can produce a variety of proteins and that two of these are extremely toxic to nerve cells. 

The study was published in the journal Science Express.