New prion protein may offer insight into mad cow disease

August 16, 2007

Scientists have discovered a new protein that may offer fresh insights into brain function in mad cow disease. “Our team has defined a second prion protein called ‘Shadoo’, that exists in addition to the well-known prion protein called ‘PrP’ ” said Professor David Westaway, director of the Centre for Prions and Protein Folding Diseases at the University of Alberta.

“For decades we believed PrP was a unique nerve protein that folded into an abnormal shape and caused prion disease: end of story. This view is no longer accurate,” Westaway adds.

The study was conducted jointly by the University of Toronto, University of Alberta, Case Western Reserve University (Ohio) and the McLaughlin Research Institute (Montana). The research is published today in the EMBO Journal and represents a culmination of work initiated at the University of Toronto in 1999, and then continued more recently at the University of Alberta.

This is the first discovery since 1985 of a new brain prion protein. “A second prion protein had been inferred by other research, based on indirect studies and the examination of DNA sequences,” said lead author Joel Watts, a graduate student at the University of Toronto’s Centre for Research in Neurodegenerative Diseases. “But we not only demonstrate that this theoretical protein really exists and shares several properties with healthy PrP; we have also defined an unexpected alteration in prion infections.

“As the PrP molecule alters shape and accumulates in a prion-affected brain, the Shadoo protein seems to disappear,” Watts added. Since proteins in a living cell are the molecules “that do the work, this is likely to be significant,” he said.

“Many facets of a prion disease like BSE are puzzling,” Westaway said. “The puzzles include the cause of death of brain cells, the function of normal prion proteins, and the rules governing emergence and spread of prions from animal to animal. We believe the Shadoo protein can give us a fresh purchase on these important questions.”

Source: University of Alberta

Explore further: Researchers find infectious prions in Creutzfeldt-Jakob disease patient skin

Related Stories

Researchers find infectious prions in Creutzfeldt-Jakob disease patient skin

November 22, 2017
Creutzfeldt-Jakob disease (CJD)—the human equivalent of mad cow disease—is caused by rogue, misfolded protein aggregates termed prions, which are infectious and cause fatal damages in the patient's brain. CJD patients ...

Brain cell advance brings hope for Creutzfeldt-Jakob disease

November 20, 2017
Scientists have developed a new system to study Creutzfeldt-Jakob disease in the laboratory, paving the way for research to find treatments for the fatal brain disorder.

Researchers observe new forms of expression of the variant of Creutzfeldt-Jakob disease

November 7, 2017
A team of French researchers from the CEA, AP-HP, CNRS, Inserm, Inra, ENVT, Institut Pasteur and MacoPharma have demonstrated that multiple variants of prions can coexist and manifest themselves in different clinical forms ...

Protein research could help in hunt for Alzheimer's and Parkinson's cures

September 11, 2017
Research carried out at the University of Kent has the potential to influence the future search for treatment of neurodegenerative diseases that are linked to a family of protein molecules known as 'amyloid'.

The flexible tail of the prion protein poisons brain cells

July 31, 2013
For decades, there has been no answer to the question of why the altered prion protein is poisonous to brain cells. Neuropathologists from the University of Zurich and University Hospital Zurich have now shown that it is ...

Cellular stress can induce yeast to promote prion formation

July 23, 2011
It's a chicken and egg question. Where do the infectious protein particles called prions come from? Essentially clumps of misfolded proteins, prions cause neurodegenerative disorders, such as mad cow/Creutzfeld-Jakob disease, ...

Recommended for you

Study opens new avenue in quest to develop tuberculosis vaccine

November 24, 2017
A team of scientists led by the University of Southampton has taken an important step forward in research efforts that could one day lead to an effective vaccine against the world's deadliest infectious disease.

Four simple tests could help GPs spot pneumonia and reduce unnecessary antibiotics

November 23, 2017
Testing for fever, high pulse rate, crackly breath sounds, and low oxygen levels could be key to helping GPs distinguish pneumonia from less serious infections, according to a large study published in the European Respiratory ...

New approach to tracking how deadly 'superbugs' travel could slow their spread

November 22, 2017
Killer bacteria - ones that have out-evolved our best antibiotics—may not go away anytime soon. But a new approach to tracking their spread could eventually give us a fighting chance to keep their death toll down.

Research points to diagnostic test for top cause of liver transplant in kids

November 22, 2017
Biliary atresia is the most common cause of liver transplants for children in the United States. Now researchers report in Science Translational Medicine finding a strong biomarker candidate that could be used for earlier ...

Alcohol consumption and metabolic factors act together to increase the risk of severe liver disease

November 22, 2017
A new study provides insights into the interaction between alcohol consumption and metabolic factors in predicting severe liver disease in the general population. The findings, which are published in Hepatology, indicate ...

Metabolites altered in chronic kidney disease

November 22, 2017
Chronic kidney disease (CKD) affects 1 in 7 people in the United States, according to the U.S. National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK). These individuals have a very high risk of cardiovascular ...

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.