The cellular prion protein (PrPC) is a glycosylphosphatidylinositol (GPI)-anchored glycoprotein encoded by the PRNP gene and predominantly expressed in neurons, although it is also found in other tissues such as lymphoid organs and the heart. PrPC has a flexible N-terminal tail and a structured C-terminal core, allowing it to interact with a wide range of partners. Its proposed physiological functions include ion balance homeostasis, neuritogenesis, neuronal protection against oxidative stress, and modulation of cell signaling pathways. However, only its role in peripheral nerve myelination homeostasis has been definitively confirmed. Misfolding of PrPC into the pathogenic PrPSc isoform leads to prion diseases, which are characterized by neurodegeneration.
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