Two top cilia researchers dedicated to ending PKD honoured with Lillian Kaplan PKD Prize
We congratuate the 2015 recipients of the Lillian Jean Kaplan International Prize for Advancement in the Understanding of Polycystic Kidney Disease (PKD): Dr Peter Igarashi and Dr Gregory J. Pazour.
The doctors received their prize in person at the World Congress of Nephrology in Cape Town in March 2015. In addition to the $50,000 cash prize, a citation depicting the winner's work and a Steuben crystal sculpture was presented.
The Lillian Jean Kaplan International Prize for Advancement in the Understanding of Polycystic Kidney Disease (PKD) is a partnership between the PKD Foundation US and International Society of Nephrology (ISN). It recognizes a medical professional or researcher exhibiting excellence and leadership in PKD research and whose work demonstrates tangible achievement toward improving knowledge and treatment of PKD.
The PKD Foundation and ISN established the award in 2002 through the generosity of Thomas Kaplan, in memory of his mother, Lillian Jean Kaplan, who had PKD and died in 2002. The award is to stimulate members of the global scientific and medical communities to increase or begin research leading to a PKD treatment and cure, generate momentum in the PKD field and produce positive public awareness about PKD. The awards presentation took place at the ISN World Congress of Nephrology in Cape Town on March 15.
Peter Igarashi, M.D.
Dr. Igarashi is the Nesbitt Chair and Head of the Department of Medicine at the University of Minnesota Medical School. He previously served as chief of the Nephrology Division at the University of Texas Southwestern Medical Center, where much of his work on PKD was conducted.
Dr. Igarashi has 30 years of research experience in kidney development, stem cells and PKD. His laboratory has identified new proteins that control genes and characterized their roles in cystic kidney disease. In addition, Dr. Igarashi has studied the role of the primary cilium in the progression of PKD. His group demonstrated that inactivation of a gene needed for the production of the primary cilium causes polycystic kidneys. Recent studies from his laboratory have revealed that non-protein coding genes play a role in PKD and represent potential therapeutic targets.
He received his bachelor's degree from the University of California, Riverside, and his M.D. degree from the University of California, Los Angeles School of Medicine. He completed residency training in internal medicine at the University of California, Davis Medical Center and a nephrology fellowship at the Yale University School of Medicine.
Gregory J. Pazour, Ph.D.
Gregory J. Pazour, Ph.D., is a professor of molecular medicine at the University of Massachusetts Medical School where he is working to understand the function of the primary cilium in mammalian development and disease. Dr. Pazour has a broad background in cilia. His postdoctoral work focused on the function and assembly mechanisms of cilia in the green alga Chlamydomonas. Work with colleagues George Witman, Joel Rosenbaum and Doug Cole demonstrated that the intraflagellar transport (IFT) system was critical for ciliary assembly.
Unexpectedly, they found that the Tg737 polycystic kidney disease gene encoded the intraflagellar transport protein IFT88 and showed that this gene is required for ciliary assembly in both Chlamydomonas and in the mouse kidney. This was the first demonstration of a ciliary defect causing polycystic kidney disease. He later went on to show that polycystin-2, the protein encoded by the human autosomal dominant PKD2 gene, is localized to cilia. These studies laid the groundwork for our current appreciation of the role of cilia in polycystic kidney disease and helped establish that the primary cilium is a sensory organelle. His laboratory is currently focused on understanding the functions of the intraflagellar transport proteins using the mouse model. This work established that cilia play critical roles in many organs including the heart and eye.
Dr. Pazour earned bachelors' degrees in chemistry and biology from South Dakota State University and a Ph.D. in biochemistry from the University of Minnesota. He did postdoctoral work at the Worcester Foundation for Biomedical Research.
PKD causes cysts to grow in the kidneys, eventually leading to kidney failure. Once a person has kidney failure, dialysis or a transplant are the only options to treat the damage the disease has caused. Parents with the disease have a 50 percent chance of passing it on to each of their children. Approximately 10 percent of the people diagnosed with PKD have no family history of the disease, with PKD developing as a spontaneous (new) mutation. Once they have it, they have a 50 percent chance of passing it on to each of their children. A relatively rare form of PKD, autosomal recessive polycystic kidney disease (ARPKD), affects approximately one in 20,000 children, and often causes death in the first month of life. Those impacted by PKD are in urgent need of treatments and a cure.