Ottawa study reveals “normal” form of the prion protein associated with neurodegenerative diseases could have role in blood sugar regulation

December 4, 2006

Diabetes researchers at the Ottawa Hospital Research Institute (OHRI) have revealed that the normal form of the prion protein that is associated with rare neurodegenerative diseases may be involved in blood sugar regulation. The OHRI is an affiliated institute of the University of Ottawa and the research arm of The Ottawa Hospital.

The findings were published today in the online edition of the scientific journal Laboratory Investigation. The upcoming print edition of the journal will feature the research as the cover story. The discovery is the result of recent work by Postdoctoral Fellow Dr. Alexander Strom, Research Associate Dr. Gen-Sheng Wang, Senior Scientist Dr. Fraser Scott and collaborators from Simon Fraser University in Burnaby and the Heinrich-Pette-Institute in Hamburg.

Prion diseases such as Creutzfeldt-Jakob disease in humans are accompanied by the accumulation of misfolded “prion” protein in the brain. The function of the misfolded prion protein has been studied extensively. However, very little is known about the role of the normal protein, which occurs naturally in many cell types, and is still described as a “black box”. The study published today, using rats as a model, is the first to suggest a connection to blood sugar control.

Dr. Scott’s research team found that normally folded prion protein can accumulate in unique aggregates inside the specialized cells of the pancreas that are responsible for producing insulin to regulate blood sugar. They also found three times more insulin-producing cells with prion protein aggregates in diabetes-prone rats. Finally, they show that the pattern of prion protein accumulation in the normal rat pancreas dramatically changes within one to three days of administering high levels of sugar into the blood.

Dr. Scott’s team is well-known for their work in environmental modification of type 1 or juvenile diabetes, a disease that occurs when the immune system attacks the insulin-producing cells in the pancreas. Recent research has focused on trying to determine what about these cells makes them susceptible to immune attack in some people. Dr. Scott cautions that although their results reveal prion protein aggregation to be a unique factor in these cells, further studies will be required to confirm the finding and determine its importance.

“At this stage, we can only speculate about the significance of the results in terms of normal body function and disease,” said Dr. Scott. “We hope that our findings will stimulate more scientific discussion and research.”

A number of neurodegenerative diseases have been associated with protein aggregation. OHRI Associate Scientist Dr. John Woulfe studies these diseases, and thinks the results published today are likely to generate a lot of interest in the neuroscience community.

“Brain cells and insulin-producing cells in the pancreas have much in common and this fascinating work provides yet another link between them. Although the work is at an early stage, the potential implications are multiple. In addition to elucidating a role for prion protein in normal glucose regulation and perhaps in diabetes, this work could have implications for understanding the cause of prion diseases,” said Dr. Woulfe, who is also a neuropathologist at The Ottawa Hospital and an Assistant Professor at the University of Ottawa.

This research was funded by the Canadian Institutes of Health Research, the Canadian Diabetes Association, and the Juvenile Diabetes Research Foundation. The OHRI is supported by The Ottawa Hospital Foundation. For more information see

Media Contact:
Jennifer Paterson
Director, Communications and Public Relations
Ottawa Hospital Research Institute
Tel: 613-798-5555 x 19691