Physiology of Cellular Prion Proteins in Reproduction.

Cellular prion protein (PrP C ) encoded at Prnp gene is well-known to form a misfolded isoform, termed scrapie PrP (PrP SC ) that cause transmissible degenerative diseases in central nervous system. The physiological role of PrP C has been proposed by many studies, showing that PrP C interacts with various intracellular, membrane, and extracellular molecules including mitochondrial inner membrane as a scaffold. PrP C is expressed in most cell types including reproductive organs. Numerous studies using PrP C knockout rodent models found no obvious phenotypic changes, in particular the clear phenotypes in development and reproduction have not demonstrated in these knockout models. However, various roles of PrP C have been evaluated at the cellular levels. In this review, we summarized the known roles of PrP C in various cell types and tissues with a special emphasis on those involved in reproduction.