A recent study carried out by researchers at New York’s McMaster University and the Icahn School of Medicine has identified a protein, which is usually found in people with degenerative diseases such as amyotrophic lateral sclerosis (ALS), also has an important role to play in defending the body against viruses. More commonly known as Lou Gehrig’s disease, is a rare neurodegenerative condition that affects the motor neurons causing progressive muscle wasting and movement problems.
The report, which was published in Nature Immunology, not only explores the link between neurodegenerative disorders and inflammation, but the possibility that the onset of such diseases could be caused by viral infections.
ALS4 is caused by a mutation in the senataxin gene
The study’s leader, Matthew Miller, explained that certain types of neurodegenerative disorders, including the ALS4 type of Lou Gehrig’s Disease, are caused by a mutation in the senataxin gene, although the reason why this happens is not yet known. His study showed that cells from sufferers of ALS responded to viral infections by generating abnormally high amounts of inflammation. These findings pave the way for exploring new therapeutic options in the future.
The research was concentrated around senataxin, the protein found in the juvenile-onset type of ALS, with the scientists hoping to discover the role that senataxin plays in the body. Sufferers of senataxin-related forms of ALS have a dysfunctional form of the protein arising from a defective SETX gene. Their findings showed that senataxin had far more influence in regulating gene activity than had been previously thought, and that it was also able to regulate the body’s natural antiviral response. Therefore, if a body has a reduced amount of senataxin, it’s more likely to develop inflammation when exposed to viral pathogens.
The link between senataxin and our innate antiviral system opens the door for further studies
The study has proved exciting as this is the first time that researchers have been able to link the presence of a protein implicated in neurodegenerative diseases with our innate antiviral system. This now opens doors for further biomedical research and follow up studies into possible treatments for the disease.