Prion disease - The propagation of infectious protein topologies

Microbes and Infection

Volumn 2, Issue 12, 2000, Pages 1445-1449

  Jackson, Graham S ^a   Collinge, John ^a  

^a IMPERIAL COLLEGE SCHOOL OF MEDICINE   (United Kingdom)

Author keywords

Prion diseases; Protein conformation; Structure activity relationship

Indexed keywords

PRION PROTEIN;

ISOMER; MOUSE; NONHUMAN; PRION; PRION DISEASE; PRIORITY JOURNAL; PROTEIN CONFORMATION; PROTEIN FOLDING; PROTEIN STABILITY; REVIEW; STRUCTURE ACTIVITY RELATION; VIRUS STRAIN;

MAMMALIA;

EID: 0033767820     PISSN: 12864579     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1286-4579(00)01299-5     Document Type: Review

References (41)

1. Prions
2. Scrapie prion protein contains a phosphatidylinositol glycolipid
3. Molecular biology of prion diseases
4. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing
5. Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins
6. Scrapie strain variation and mutation
7. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
8. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
9. Mice devoid of PrP are resistant to scrapie
10. Homozygous prion protein genotype predisposes to sporadic Creutzfeldt-Jakob disease
11. Mutations and polymorphisms in the prion protein gene
12. Genetic predisposition to iatrogenic Creutzfeldt-Jakob disease
13. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie priori replication
14. Quantifying the kinetic parameters of prion replication
15. Cell-free formation of protease-resistant prion protein
16. Non-genetic propagation of strain-specific properties of scrapie prion protein
17. Protease-resistant prion protein produced in vitro lacks detectable infectivity
18. NMR structure of the mouse prion protein domain PrP (121-231)
19. Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform
20. Structural mobility of the human prion protein probed by backbone hydrogen exchange
21. Extremely rapid folding of the C-terminal domain of the prion protein without kinetic intermediates
22. Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein
23. Proteins can adopt totally different folded conformations
24. Autonomous and reversible folding of a soluble amino-terminally truncated segment of the mouse prion protein
25. Multiple folding pathways for heterologously expressed human prion protein
26. pH-dependent stability and conformation of the recombinant human prion protein PrP (90-231)
27. A scrapie-like unfolding intermediate of the prion protein domain PrP(121-231) induced by acidic pH
28. Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations
29. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform
30. Eight prion strains PrP(Sc) molecules with different conformations
31. Neurotoxicity of a prion protein fragment
32. Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity
33. The cellular prion protein (PrP) selectively binds to Bcl-2 in the yeast two-hybrid system
34. Analysis of interaction sites in homo- and heteromeric complexes containing Bcl-2 family members and the cellular prion protein
35. PrP deposition, microglial activation, and neuronal apoptosis in murine scrapie
36. Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions
37. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein Doppel
38. Recombinant scrapie-like prion protein of 106 amino acids is soluble
39. Prion protein of 106 residues creates an artificial transmission barrier for prion replication in transgenic mice
40. Normal host prion protein necessary for scrapie-induced neurotoxicity
41. Strain-specific prion-protein conformation determined by metal ions