PRNP

gene

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July 20, 2026

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The PRNP gene encodes for the prion protein (PrP). This protein is normally found on the surface of neurons in the brain and spinal cord. However, prions can arise from mutations in the PRNP gene or through infection.12

When the prion protein misfolds into an abnormal shape, it resists breakdown and accumulates in neurons, leading to their destruction. This process creates sponge-like holes in brain tissue, causing fatal neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs). Examples of human prion diseases include Creutzfeldt-Jakob disease and fatal familial insomnia, while animal diseases include bovine spongiform encephalopathy (mad cow disease). Unlike other disease agents, prions do not appear to contain DNA or RNA.123

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Creutzfeldt-Jakob disease
Types
…mutation in a gene designated PRNP), which encodes the prion protein PrP, is passed from parent to child in a dominant fashion (i.e., only one of the two copies of the gene that are inherited—one from each parent—need be mutated for disease to occur). More than 50 different mutations in…
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…mutation in a gene designated PRNP), which encodes the prion protein PrP, is passed from parent to child in a dominant fashion (i.e., only one of the two copies of the gene that are inherited—one from each parent—need be mutated for disease to occur). More than 50 different mutations in…
Read More