The National Prion Disease Pathology Surveillance Center based at Case Western Reserve University's School of Medicine has received renewed grants amounting to up to more than $27.5 million over the next five years to fund the continual efforts of the center, where any suspected case of prion disease—such as Creutzfeldt-Jakob disease (CJD)—is reported, characterized and tested, as well as prion research. The grants were awarded by the Centers for Disease Control and Prevention (CDC) and the National Institute on Aging (NIA), an arm of the National Institutes of Health (NIH).
Pierluigi Gambetti, center director, department of pathology, Case Western Reserve University, professor of pathology and neurology at the School of Medicine and University Hospitals Case Medical Center, is the principal investigator on all CJD research and diagnostic efforts. Other Case Western Reserve participants in the grants are Witold Surewicz, professor of physiology and biophysics and co-principal investigator; Qingzhong Kong and Wen-Quan Zou, assistant professors of pathology. Claudio Soto, professor of neurology and director of the George and Cynthia Mitchell Center for Alzheimer's Disease Research at the University of Texas Medical Branch, Galveston, is also a project leader in the grant.
Each year the center diagnoses and reports the growing number of cases of CJD to the CDC and federal government. "Mad cow," CJD and variant CJD (vCJD) are prion diseases, caused when prion proteins become abnormal. These abnormal prions are deadly infectious agents that, in turn, corrupt healthy prions. Incidence of the disease is one case per million people. Most victims of prion-related diseases are over the age of 65, leading scientists to believe that sporadic CJD is a disease related to the aging process, possibly triggered when the cellular protein-making mechanism spontaneously goes awry, Gambetti explained.
"It is exciting that the National Prion Disease Pathology Surveillance Center is based here," said Gambetti, who for more than 15 years has been acknowledged as one of the foremost researchers of prion-related diseases. "It is even more important that there is a growing awareness of prion diseases thanks to the center's efforts in encouraging reporting by physicians and patients' families nationwide, partially by cultivating relationships with each state's departments of health, as well as close collaboration with the CJD Foundation."
Back in the early 1990s, when Britain's mad cow frenzy was in full swing, Gambetti and his team of researchers became widely known when they showed that a rare disease called fatal familial insomnia was caused by rogue prions. The recent work of Surewicz and his coworkers has provided critical support to the prion hypothesis and to the understanding of prion-strain characteristics. Prion proteins are normally found throughout the body attached to the outside of human cells, but become pathogenic (disease-causing) through a change in conformation.
The NIA award will allow Gambetti and the other researchers associated with the center to focus on identifying the mechanisms that lead to the formation of distinct species or strains which, in turn, determine different characteristics in human prion diseases. In addition, the awards will help fund the development of new diagnostic methods as well as the research program directed at the characterization of the existing prion diseases and the detection of novel prion diseases.
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