Prion Protein (朊病毒蛋白)

Prion Protein is a protein that is converted from a normal cell protein (PrPC) to a pathogenic conformation (PrPSc). PrPC is an α-helices rich membrane glycoprotein, which regulates neuronal development, synaptic function, intercellular signaling, cellular oxidative stress response, and apoptosis under normal physiological conditions. PrPC is converted into β-folding rich PrPSc under pathological conditions, leading to neuronal damage and death. Prion Protein dysfunction and misfolding causes prion diseases such as Creutzfeldt-Jakob Disease (CJD), Fatal Familial Insomnia (FFI), Gerstmann-Sträussler-Scheinker Syndrome (GSS), and other neurodegenerative diseases such as Alzheimer's Disease (AD), Parkinson's Disease (PD) or Multiple System Atrophy (MSA)^[1].