Human prion protein

The human prion protein (PrP) is a naturally occurring protein found mainly in the brain and nervous system. Its normal role involves helping protect nerve cells and possibly facilitating cell signaling. However, when this protein misfolds into an abnormal shape, it can become infectious, triggering the misfolding of other prion proteins. These abnormal proteins accumulate and cause brain damage, leading to neurodegenerative diseases like Creutzfeldt-Jakob disease. The precise functions of the normal prion protein are still being studied, but its role in brain health and disease highlights the importance of proper protein structure in maintaining neurological function.