U of A-Led Research Suggests New Culprit in Mad Cow Disease

Groundbreaking research led by the University of Alberta challenges the belief that mad cow disease is caused only by misfolded proteins — a discovery that sheds new light on the devastating outbreak in the United Kingdom 40 years ago and provides new hope for prevention.

The study shows for the first time that such prion-like brain diseases can be triggered without the presence of infectious prions. Prion disease occurs when normal proteins in the brain misfold into infectious, abnormal proteins.

Instead, chronic inflammation caused by a powerful bacterial endotoxin called lipopolysaccharide (LPS) was identified as a culprit that can independently trigger brain damage resembling prion disease.

“This fundamentally challenges the prevailing theory that these types of brain diseases are only about prions or similar misfolded proteins,” says Burim Ametaj, a nutritional immunobiologist in the Faculty of Agricultural, Life & Environmental Sciences and lead author on the study.