Prion disease may be caused by buildup of cellular trash, say Stanford researchers

STANFORD, Calif. - Mutant mice whose brains gradually become peppered with small holes resembling those found in prion disease lack a protein involved in disposing of cellular trash, say researchers at Stanford University Medical Center. The finding may shed light on how diseases such as Creutzfeldt-Jakob, bovine spongiform encephalopathy (also known as mad cow disease), and scrapie wreak havoc in humans and other mammals, and lends further support to the growing notion that glitches in protein turnover may be the unifying element in many neurodegenerative disorders.

Although the scientists caution that the mutant mice don't accumulate the misfolded protein, or prion, associated with the infectious forms of spongiform encephalopathy, the rodents' brains are dead-ringers for the brains of people and cows who have died from the disease. They speculate that the mutation in a ubiquitin ligase - a protein that flags other proteins for destruction by the cell's recycling machinery - may represent a downstream step in the cascade of events that leaves the brain looking somewhat like a kitchen sponge.

"No one really understands how or why spongy degeneration develops," said Gregory Barsh, MD, PhD, professor of pediatrics and of genetics in the Stanford School of Medicine, in whose laboratory the research was conducted. "Now we have a molecular handle with which to study it." The research is published in the Jan. 31 issue of Science.

Ubiquitin ligases are just a few members of a complex team of proteins that make up the ubiquitin pathway. Together they identify and physically tag abnormal, misfolded or simply worn-out proteins for dismantling in the cell's recycling center. Until recently they were about as glamorous as garbage collectors.

The study of neurodegenerative diseases such as Parkinson's, Huntington's and Lou Gehrig's upped the ubiquitin ante, however, with the discovery that patients' brain cells share a common trait: large clumps

Contact: Krista Conger
Stanford University Medical Center

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