CD230 or major prion protein (PrP)

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Function

Structure

  • glypiated cell-surface glycoprotein
  • can occur in 2 structural conformations:
    • PrPc (normal, of unknown function) C-terminal domain folded mostly in alpha-helical conformaion (3 alpha helices & a short antiparallel beta-sheet), stabilized by single disulfide bond linking helices 2 & 3
    • PrPSc (abnormal protein resulting in disease) PrPSc has high beta-sheet content

Compartment

Expression

  • present in most mammalian tissues
  • highest levels in CNS
  • widely expressed in cells of immune system

Pathology

Polymorphism

Laboratory

Analysis:[2]

More general terms

Additional terms

References

  1. Stedman's Medical Dictionary 27th ed, Williams & Wilkins, Baltimore, 1999
  2. 2.0 2.1 Journal Watch 21(15):125, 2001 Saborio et al Nature 411:810, 2001 Maissen et al Lancet 357:2026, 2001
  3. Johnson & Gibbs NEJM 339:1994, 1998
  4. Coughey B. Trends in the Biochemical Sciences 26:235, 2001
  5. Entrez Gene http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=5621
  6. 6.0 6.1 McLennan NF et al, Prion protein accumulation and neuroprotection in hypoxic brain damage Am J Pathol 2004, 165:227 PMID: https://www.ncbi.nlm.nih.gov/pubmed/15215178
  7. Sanchez-Juan P et al, CSF tests in the differential diagnosis of Creutzfeldt-Jakob disease. Neurology 2006, 67:637 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16924018
    Collins SJ et al, Determinants of diagnostice investigation sensitivities across the clinical spectrum of sporadic Creutzfeldt-Jakob disease Brain 2006, 129:2278 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16816392
    Cali I et al, Classification of sporadic Creutzfeldt-Jakob disease revisited. Brain 2006, 129:2266 PMID: https://www.ncbi.nlm.nih.gov/pubmed/16923954
  8. 8.0 8.1 Lauren J et al. Cellular prion protein mediates impairment of synaptic plasticity by amyloid-oligomers. Nature 2009 Feb 26; 457:1128 PMID: https://www.ncbi.nlm.nih.gov/pubmed/19242475
    Cisse M and Mucke L. Alzheimer's disease: A prion protein connection. Nature 2009 Feb 26; 457:1090. PMID: https://www.ncbi.nlm.nih.gov/pubmed/19242462
  9. 9.0 9.1 Medical Knowledge Self Assessment Program (MKSAP) 19. American College of Physicians, Philadelphia 2021

Database