Researchers discover common cause of all forms of ALS. The underlying disease process of amyotrophic lateral sclerosis (ALS and Lou Gehrig’s disease), a fatal neurodegenerative disease that paralyzes its victims, has long eluded scientists and prevented development of effective therapies. Scientists weren’t even sure all its forms actually converged into a common disease process.
Researchers from the Bellvitge Biomedical Research Institute at the University of Barcelona have coordinated research into how the IDPN nitrile causes neurological syndromes similar to those of the amyotrophic lateral sclerosis (ALS), a severe neuromuscular degenerative disease.
Scientists knew that mutations in the FUS gene (Fused in Sarcoma) cause amyotrophic lateral sclerosis (ALS), a disease of the nerve cells in the brain and spinal cord that control voluntary muscle movement. The researchers were successful in identifying mutations in this gene that cause Essential Tremor, and proved that the disease mechanisms for ET and ALS FUS mutations are different.
By increasing the signaling activity of a protein called muscle skeletal receptor tyrosine-protein kinase (MuSK), researchers were able to keep nerve cells attached to muscle longer into the progression of the disease in a mouse model of ALS.
Professional football players in this study were three times more likely to die as a result of diseases that damage brain cells compared to the general population. A player’s risk of death from Alzheimer’s disease or ALS was almost four times higher than the general population.
While evidence suggests pathological proteins linked to the onset and progression of neurodegenerative disorders are capable of spreading from cell-to-cell within the brains of affected individuals, new research shows no evidence to support concerns that these abnormal disease proteins are “infectious” or transmitted from animals to humans or from one person to another.