Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD.
Strains of transmissible spongiform encephalopathies are distinguished by differing physicochemical properties of PrPSc, the disease-related isoform of prion protein, which can be maintained on transmission to transgenic mice. 'New variant' Creutzfeldt-Jakob disease (CJD) has strain characteristics distinct from other types of CJD and which resemble those of BSE transmitted to mice, domestic cat and macaque, consistent with BSE being the source of this new disease. Strain characteristics revealed here suggest that the prion protein may itself encode disease phenotype.
Collinge
J
J
Neurogenetics Unit, Department of Biochemistry and Molecular Genetics, Imperial College School of Medicine at St. Mary's, London, UK. j.collinge@ic.ac.uk
Sidle
K C
KC
Meads
J
J
Ironside
J
J
Hill
A F
AF
D000818:Animals; D002415:Cats; D007562:Creutzfeldt-Jakob Syndrome / Q000145:classification / Q000209:etiology* / Q000635:transmission; D016643:Encephalopathy, Bovine Spongiform / Q000209:etiology; D019286:Endopeptidase K / Q000378:metabolism; D014644:Genetic Variation; D006031:Glycosylation; D013006:Growth Hormone / Q000008:administration & dosage; D006801:Humans; D008251:Macaca; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D018621:PrPC Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D018620:PrPSc Proteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism; D011328:Prions / Q000737:chemistry / Q000145:classification / Q000235:genetics* / Q000378:metabolism; D011487:Protein Conformation
D018621:PrPC Proteins; D018620:PrPSc Proteins; D011328:Prions; D013006:Growth Hormone; D019286:Endopeptidase K