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November 2006
Children's Research Institute News Brief
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Bernard L. Maria, MD, MBA
Executive Director
Darby Children's
Research Inst. |
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Inderjit Singh, PhD
Scientific Director
Darby Children's
Research Inst. |
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DCRI Adds New Researcher with Expertise in Polycystic Kidney Disease
Excited about the Charles P. Darby Children's Research Institute's commitment to basic research, scientist Dr. Darwin Bell 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 urging, and felt really excited about what's going on, about the emphasis on growth and new research,"
says Dr. Bell, a pediatric nephrologist. "It really appealed to me to become part of what I see as a medical school on the rise."
Dr. 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.
"It's a very prevalent disease which results in kidney failure," explains Dr. Bell.
Polycystic means multiple cysts; with PKD, there are multiple cysts on each kidney. Growing and multiplying over time, these cysts cause the kidney
to enlarge. Ultimately the diseased kidney shuts down, making dialysis and transplantation the only forms of treatment.
Dr. Bell's lab 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," says Dr. Bell.
"We're hoping to identify how the loss of cilia structure affects function, how it causes cysts to be formed in the kidney," he continues.
The disease has two forms - autosomal dominant PKD is the most common, affecting about one in 500 adults. Recessive PKD is less common, but affects newborns,
infants and children, having a devastating effect on kidneys and other organs.
Dr. Bell's lab aims 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, Dr. Bell says 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," says Dr. Bell. "I'm excited about the opportunity to collaborate with other scientists on other diseases that affect children."
In the future, he anticipates working with Dr. Bernie Maria 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."
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