Dr. Fred Goldman, Department of Pediatrics, and Dr. Al Klingelhutz, Department of Microbiology have teamed up with researchers from the University of Vancouver, British Columbia, on a project to study how abnormalities in an enzyme called telomerase lead to defects in immune response and hematopoiesis (generation of blood cells). Telomerase is the cellular enzyme responsible for maintaining telomeres, the protective structures at the ends of chromosomes. Dr. Goldman’s team has played a significant role in identifying and characterizing an inherited disease called Dyskeratosis congenita (DC), which is due to a telomerase defect. DC results in bone marrow failure and a premature aging syndrome. Dr. Klingelhutz has a longtime interest in studying the role of telomerase in cell immortalization and cancer.
The two UI researchers will receive approximately $400,000 over the next 5 yeas as part of collaboration with Dr. Peter Lansdorp, University of Vancouver, who is heading a Program Project being funded by the Canadian Institutes of Health Research. The goal of the research team will be to develop methodologies to correct the telomerase defect in DC cells and to determine how telomerase might play a role in other diseases associated with lymphocyte and hematopoietic stem cell malfunction.
Normal megakaryocytes |
Transfected megakaryocytes |
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