MONDAY, July 10 (HealthDay News) -- Researchers report the development of a potential new diagnostic tool and mouse model for studying Creutzfeldt-Jakob disease (CJD) that will hopefully assist in the development of badly needed human blood tests for CJD, according to two studies in the July 7 issue of Science.
The misfolded version of the prion protein that is responsible for producing CJD symptoms is difficult to detect during the presymptomatic phase of infection and may pose a formidable health problem due to undetected blood transmission. Paula Saa, from the University of Texas Medical Branch in Galveston, and colleagues show that protein misfolding cyclic amplification (PMCA) technology can be used to detect prion proteins in the presymptomatic phase in hamsters, perhaps even when only a single infectious unit is present.
In the second study, Matthew J. Trifilo, Ph.D., from the Scripps Research Institute in La Jolla, Calif., and colleagues report the development of a transgenic mouse model of CJD that reliably produces infectious prion proteins at detectable levels in the blood. Interestingly, these mice seem to develop heart disease, instead of neurological problems, due to deposits of prion proteins in the heart.
Future studies could test the possibility that cardiac deposition occurs in humans, Trifilo and colleagues conclude, but in the meantime their mouse could be used to test the sensitivity of other new diagnostic tests, perhaps even the one presented by Saa, and to test new treatments.
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