Discovery may revolutionize ALS treatment

VANCOUVER, British Columbia, Sept. 21 (UPI) -- A link between prions and the neurodegenerative disease amyotrophic lateral sclerosis might lead to new treatments, Canadian researchers say.

Dr. Neil Cashman, academic director of the Vancouver Coastal Health ALS Center, and colleagues at the University of British Columbia, said the study demonstrates that the SOD1 protein -- which has been shown to be implicated in the ALS disease process -- exhibits prion-like properties. A prion is an infectious agent composed of protein in a misfolded form.

The researchers found that SOD1 participates in a process called template-directed misfolding, which refers to the coercion of one protein by another protein to change shape and accumulate in large complexes in a fashion similar to the process underlying prion diseases.

The discovery is significant as it opens the door to novel approaches to the treatment of ALS -- also known as Lou Gehrig's disease -- Cashman said.

"Our work has identified a specific molecular target, which when manipulated halts the conversion of the SOD1 protein to a misfolded, disease-causing form," Cashman said in a statement. "This discovery is a first-step toward the development of targeted treatments that may stop progression of ALS."

The study was published in the Proceedings of the National Academy of Sciences.