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Ironing your genes: Thalassemia grant 

Pawelek leads study into thalassemia

By Anna Sarkissian

With major research grants, high profile conferences and cutting-edge facilities, it’s clear that Concordia is making a mark on Canadian scientific research.

Assistant professor Peter Pawelek from the Department of Chemistry and Biochemistry was recently awarded a two-year grant from the Thalassemia Foundation of Canada and is very pleased with the way the university has facilitated his work.

“I’m excited to see where this research will take Concordia in terms of opening doors for the development of therapeutics,” he said.

Pawelek’s new funds will go towards research into cell-surface receptors and pathogens, which could help patients with thalassemia, a blood disease that is prevalent in Mediterranean, Arab and Asian populations.

The genetic condition leads to a deficiency in hemoglobin, which normally binds iron. This causes excess iron to build up in the bloodstream, which in turn can cause tissue and organ damage and other complications.

“Usually pathogens have difficulty obtaining iron from a host,” Pawelek explained. “When that limiting factor is taken away and you have too much iron, patients are more at risk from bacterial infections.”

One of the therapies to treat thalassemia involves iron chelation, in which a drug such as Desferal is administered through a transfusion to bind iron and remove it from the body.

However, some highly virulent and lethal bacteria, such as Vibrio vulnificus, have protein receptors that can recognize and take up iron-bound Desferal, hijacking the drug and compounding the risk of infection.

“If we can understand the shape of the binding pocket of the receptor, we could one day be able to design a molecule to fit in that receptor, acting as a kind of monkey wrench to throw into the system,” Pawelek said.

The first step will be to isolate the cell-surface receptor, purify it and grow crystals of it. Next, Pawelek’s group will be able to characterize the three-dimensional protein structure using X-ray crystallography.

Having a better understanding of how bacteria manipulates Desferal can have implications beyond thalassemia since it is widely prescribed for other iron-overload diseases and some forms of cancer.

“We’re hoping that our work will lay the foundation for us to move forward and continue this research beyond two years,” he said. “And ultimately produce a therapeutic agent that can help people.”

Pawelek also directs a separate NSERC-funded research program that investigates how bacteria use molecules called siderophores to acquire iron. This research recently led to an article published in the Journal of Molecular Biology.

He is also busy organizing the tenth annual PROTEO symposium at Concordia in May, which will bring together protein scientists from the Quebec Research Network on Protein Function, Structure and Engineering. Internationally renowned researchers have also been invited to present their work.

“Concordia is really on the map now in terms of being a meaningful presence in the biosciences community,” he said.