Mayo Clinic Researchers Discover Toxic Process that Leads to Dementia, ALS

Researchers at Mayo Clinic in Florida have uncovered a toxic cellular process by which a protein that maintains the health of neurons becomes deficient and can lead to dementia. The findings shed new light on the link between culprits implicated in two devastating neurological diseases: frontotemporal dementia and amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig’s disease. The study appears in the Dec. 10 online issue of Proceedings of the National Academy of Sciences.

There is no cure for frontotemporal dementia — a disorder that affects personality, behavior and language, and is second only to Alzheimer’s disease as the most common form of early-onset dementia. While much research is devoted to understanding the role each defective protein plays in these diseases, the team at Mayo Clinic took a new approach to examine the interplay between TDP-43, a protein that regulates messenger ribonucleic acid (mRNA) — biological molecules that carry the information of genes and are used by cells to guide protein synthesis — and sortilin, which regulates the protein progranulin. For more information, click here to read the full release.