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DCRI researcher adds expertise in disease Excited about Charles P. Darby Children’s Research Institute’s (DCRI) commitment to basic research, scientist and pediatric nephrologist Darwin Bell, M.D., recently relocated from the University of Alabama at Birmingham to set up his lab in the DCRI.   “I had visited the DCRI at Dr. David Ploth’s [nephrology] urging, and felt really excited about what’s going on, about the emphasis on growth and new research,” Bell said. “It really appealed to me to become part of what I see as a medical school on the rise.”   Bell’s lab in the DCRI focuses on polycystic kidney disease (PKD), the most common genetic, life-threatening disease. It affects more than 600,000 Americans and results in kidney failure. Polycystic means there are multiple cysts on each kidney. Growing and multiplying over time, the cysts cause the kidney to enlarge. Ultimately the diseased kidney shuts down, making dialysis and transplantation the only forms of treatment. The disease has two forms, with autosomal dominant PKD as the most common and affecting about one in 500 adults. Recessive PKD is less common but affects newborns, infants and children, and has a devastating effect on kidneys and other organs.   Bell is studying the individual cells of these diseased kidneys.   “PKD is by-and-large a disease of the cilia, the fingerlike projections on the cell which act like antennas. We are trying to understand the function of cilia in cells, to grasp how these ‘antennas’ work,” Bell said. “We’re hoping to identify how the loss of cilia structure affects function, how it causes cysts to be formed in the kidney.”   Bell’s ultimate goal is to identify and show therapeutic agents and modalities that would either prevent cysts from forming in the kidney, or reduce their size and number.   Impressed with the DCRI’s resources, including new and well-planned labs and facilities, Bell said he’s challenged to pursue avenues of collaboration.   “We’re using sophisticated confocal and imaging techniques that we think have a wide application for other kinds of research, too, in terms of diseases such as hypertension,” he said. “I’m excited about the opportunity to collaborate with other scientists on other diseases that affect children.”   In the future, Bell anticipates working with Bernie Maria, M.D., DCRI executive director, to tackle Joubert’s Syndrome, another cystic disease with a neural and kidney component. “Hopefully we can find out something that would be of benefit in the next few years,” Bell said.     Friday, Nov. 24, 2006 Catalyst Online is published weekly, updated as needed and improved from time to time by the MUSC Office of Public Relations for the faculty, employees and students of the Medical University of South Carolina. Catalyst Online editor, Kim Draughn, can be reached at 792-4107 or by email, catalyst@musc.edu. Editorial copy can be submitted to Catalyst Online and to The Catalyst in print by fax, 792-6723, or by email to catalyst@musc.edu. To place an ad in The Catalyst hardcopy, call Island Publications at 849-1778, ext. 201.