Prion Proteins

**Definition:** Membrane glycosylphosphatidylinositol-anchored glycoproteins that may aggregate into rod-like structures. The prion protein (PRNP) gene is characterized by five TANDEM REPEAT SEQUENCES that encode a highly unstable protein region of five octapeptide repeats. Mutations in the repeat region and elsewhere in this gene are associated with CREUTZFELDT-JAKOB DISEASE; FATAL FAMILIAL INSOMNIA; GERSTMANN-STRAUSSLER DISEASE; Huntington disease-like 1, and KURU.

**Tree numbers:** - D09.400.430.890.448.600 - D12.776.395.550.448.600 - D12.776.543.484.500.625 - D12.776.543.550.418.600 - D12.776.785.340

**Synonyms:** - PrP Proteins - Fatal Familial Insomnia Protein - Prion Protein - CD230 Antigen - Creutzfeldt-Jakob Disease Protein - Major Prion Protein