The cellular prion protein (PrPC) is well-known for its ability to converse into its pathological isoform, PrPTSE. Accumulation of PrPTSE in the brain is associated with pathogenesis of prion diseases.
Numerous studies have suggested that PrPC has a number of physiological functions and participates in many cellular processes. However, convincing evidence is still missing.
Possible functions of PrPC include a role in regulation of apoptosis, protection against oxidative stress, cell adhesion or processes of learning and memory. This protein also seems to influence cell proliferation and differentiation.
The level of PrPC expression during embryonic development affects transcription of genes encoding factors involved in the regulation of stem cells pluripotency at early stages of differentiation. In the nervous system, PrPC plays an important role in neuronal development, maturation and neural circuit formation.
Finally, PrPC can probably also participate in the differentiation and proliferation of tissue-specific stem cells such as neuronal, hematopoietic or myogenic precursors.