VIDO researchers are investigating an untreatable disease

SASKATOON – Even Dr. Scott Napper (PhD) uses words like scary when describing the focus of his research – a group of diseases caused when otherwise harmless proteins break away, creating conditions that are always fatal and currently untreatable.

“But that’s what I love about it — it’s so interesting and so very important,” said Napper, a scientist with the Vaccines and Infectious Diseases Organization (VIDO) at the University of Saskatchewan (USask).

Napper is playing a leading role in finding vaccines to treat diseases caused by misfolded proteins. His original research focused on prion diseases, including mad cow disease, scrapie in sheep, and chronic wasting disease (CWD) in deer and elk.

However, several similar human neurodegenerative diseases are also on his radar, including Alzheimer’s, Huntington’s, Parkinson’s and amyotrophic lateral sclerosis (Lou Gehrig’s disease).

“Is CWD the priority? Yes. Is Alzheimer’s in the foreground? Yes. We are doing this work in parallel on both human and animal vaccines,” he said.

Napper explained that these diseases arise when a protein misfolds and takes on new and devastating properties. And once misfolded, the new protein becomes self-replicating.

When the misfold comes into contact with a correctly folded version of itself, that protein, too, becomes misfolded, “so that one becomes two and two becomes four, and you get a cascade of misfolds that result in a fatal disease.”

Prion diseases first attracted attention in the early 1950s with the discovery of kuru, a neurodegenerative disease found among the Fore peoples of Papua New Guinea, Napper said. It has been linked to cannibalism at funerals – the Fore people cooking and eating their deceased family members, thus spreading the disease.

When the cause of kuru was identified as a proteinaceous infectious particle, or prion, about 20 years later, “it was overwhelming,” Napper said. “It’s completely changed the way we think about infectious diseases because they’re not bacteria or viruses.”

It is now known that this prion-like mechanism is responsible for other human neurodegenerative diseases such as Alzheimer’s. And the search for a cure has recently received a boost.

The Alberta Conservation Association, in partnership with Alberta Environment and Parks and the Saskatchewan Department of Environment, has committed $1.2 million to fund research into an oral vaccine to treat CWD, which poses a significant risk to wild populations in four provinces and 28 represents states.

A team of scientists from four western Canadian universities, including Napper, Dr. Philip Griebel (DVM, PhD) and Dr. Suresh Tikoo (DVM, PhD) at VIDO and Dr. Sidney Hayes (PhD) at the College of Medicine, aim to develop oral vaccines for CWD that can be released into affected habitats. The other researchers are from the Universities of British Columbia, Alberta and Calgary.

Scientists will work with wildlife groups to develop and deliver an effective real-world solution.

“We wear lab coats and they wear camouflage, but we can all work together,” Napper said.

On the human front, the Weston Family Foundation, through the Weston Brain Institute Napper and three research colleagues at other Canadian universities, is providing $1.2 million to develop a vaccine against misfolded protein diseases in humans. Building on previous developments, scientists will generate antibodies that bind to misfolded proteins – one has already been selected for preclinical development – to prevent disease development.

In both cases, Napper appreciates the team approach.

“As we saw with COVID, when the global scientific community came together to develop a vaccine, when we work together and leave our self-interests and egos at the door, we are better, stronger and smarter.”

That’s not to say the work will be easy, but early results are very encouraging, Napper said. “The pipeline is there, and once we have the target protein, we’ve been able to get the types of immune responses we need. All of the diseases we’ve talked about have a different target protein, but the development of the vaccines all use similar processes.”

Napper, who is also a professor of biochemistry at USask College of Medicine, believes VIDO’s unique culture and expertise will make a significant contribution.

Founded in 1975 to focus on infectious diseases in the Canadian livestock industry, VIDO expanded its mission to include research and vaccine development related to human diseases, COVID-19 being an example. The breadth of talent among VIDO scientists means the organization “can tackle complex problems with a team approach,” he said.

“I’m not an immunologist, but the guy in the office next door is, and there’s a scientist just down the floor who’s been developing oral vaccines for 20 years. We all want to make the world a better place.”

Napper admitted that it’s faster to develop and commercialize a vaccine for animals than for humans, “so it’s not unreasonable to envision a CWD vaccine in seven to 10 years.” A human vaccine will take longer, though Napper is undeterred.

“Our initial success might be simply slowing the progression of the diseases, but we will take the incremental gains as they come. There is hope.”

— Submitted by USask Media Relations