New insight into prion-related diseases
EU-funded PRP AND NEURODEGENER project studied a number of aspects of the pathology involved in transmissible spongiform encephalopathies. These diseases, including CJD in human and BSE in cows are caused by protein-like components called prions (PrP). PrP is the pathogenic version of a PrPC, a 'normal' version of the prion protein occurring naturally in brain cells. The role of PrP has not been elucidated; neither that of its structurally similar Doppler (Dpl) protein. There have been indications however, that Dpl may be involved in male fertility in mice. It is hoped that understanding the physiological function of PrPC and Dpl could aid in the understanding of neurodegenerative mechanisms during transmissible spongiform encephalopathies. University of Leeds researchers focused on Dpl function in cultured cells. Studies showed that Dpl can be found in human testis and sperm cells as well as seminal plasma. PrPC is also being expressed in sperm cells. The expression pattern of Dpl suggests a possible role in male fertility in humans, although further tests would be needed before such a hypothesis could be validated. Developing new therapeutic strategies against these diseases is very much dependent on obtaining a clear view of the PrPC's and Dpl's physiological roles and their potential links. Given the economic losses incurred during the last outbreak of BSE, it is easier to obtain the size of the market for these new therapeutics and therefore the value behind such projects.
