Strain-specified relative conformational stability of the scrapie prion protein

Protein Science

Volumn 10, Issue 4, 2001, Pages 854-863

  Peretz, David ^a,b   Scott, Michael R ^a,b   Groth, Darlene ^a,b   Williamson, R Anthony ^c   Burton, Dennis R ^c   Cohen, Fred E ^a,b   Prusiner, Stanley B ^a,b  

^a UNIVERSITY OF CALIFORNIA   (United States)

^b UNIVERSITY OF CALIFORNIA   (United States)

^c SCRIPPS RESEARCH INSTITUTE   (United States)

Author keywords

Neurodegenerative disease; Prion protein; Prion strains; Protein conformation; Scrapie; Spongiform encephalopathies

Indexed keywords

GUANIDINE; PRION PROTEIN; PROTEINASE;

ARTICLE; BRAIN SPONGIOSIS; CONCENTRATION RESPONSE; CONFORMATIONAL TRANSITION; CONTROLLED STUDY; DEGENERATIVE DISEASE; HAMSTER; INCUBATION TIME; MOLECULAR STABILITY; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DEGRADATION; PROTEIN DENATURATION; STRAIN DIFFERENCE;

ANIMALS; ANTIBODIES; CRICETINAE; ENDOPEPTIDASES; ENZYME-LINKED IMMUNOSORBENT ASSAY; GUANIDINE; MESOCRICETUS; PRIONS; PROTEIN CONFORMATION; PROTEIN DENATURATION; SCRAPIE; SPECIES SPECIFICITY;

CRICETINAE; MESOCRICETUS AURATUS; SCRAPIE PRION;

EID: 0035086136     PISSN: 09618368     EISSN: None     Source Type: Journal    
DOI: 10.1110/ps.39201     Document Type: Article

References (69)

1. Principles that govern the folding of protein chains
2. Scrapie prion liposomes and rods exhibit target sizes of 55,000 Da
3. Biochemical and physical properties of the prion protein from two strains of the transmissible mink encephalopathy agent
4. Identification of two biologically distinct strains of transmissible mink encephalopathy in hamsters
5. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
6. Biological evidence that the scrapie agent has an independent genome
7. Precise targeting of the pathology of the sialoglycoprotein, PrP, and vacuolar degeneration in mouse scrapie
8. A large array of human monoclonal antibodies to type 1 human immunodeficiency virus from combinatorial libraries of asymptomatic seropositive individuals
9. The binding characteristics of proteins for polystyrene and their significance in solid-phase immunoassays
10. Linkage of prion protein and scrapie incubation time genes
11. Prion protein and the transmissible spongiform encephalopathies
12. Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy
13. Strain-dependent differences in β-sheet conformations of abnormal prion protein
14. Encephalopathy in mice produced by inoculation with scrapie brain material
15. Pathologic conformations of prion proteins
16. Identification of a gene which controls the incubation period of some strains of scrapie agent in mice
17. Neurotoxicity of a prion protein fragment
18. Transmission of bovine spongiform encephalopathy to sheep and goats
19. Neuropathology of scrapie: The precision of the lesions and their diversity
20. The sequential development of the brain lesions of scrapie in three strains of mice
21. Purified prion proteins and scrapie infectivity copartition into liposomes
22. Transmissible and non-transmissible amyloidoses: Autocatalytic post-translational conversion of host precursor proteins to β-pleated sheet configurations
23. Predicted α-helical regions of the prion protein when synthesized as peptides form amyloid
24. Perturbation of the secondary structure of the scrapie prion protein under conditions that alter infectivity
25. Transmission of sheep and goat strains of scrapie from experimentally infected cattle to hamsters and mice
26. Replication of distinct scrapie prion isolates is region specific in brains of transgenic mice and hamsters
27. Specific binding of normal prion protein to the scrapie form via a localized domain initiates its conversion to the protease-resistant state
28. Seeding "one-dimensional crystallization" of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie?
29. Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation
30. Immunological comparison of scrapie-associated fibrils isolated from animals infected with four different scrapie strains
31. Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins
32. The genomic identity of different strains of mouse scrapie is expressed in hamsters and preserved on reisolation in mice
33. Formic acid pretreatment enhances immunostaining of cerebral and systemic amyloids
34. Circular dichroism
35. Cleavage of structural proteins during the assembly of the head of bacteriophage T-4
36. Comparison of scrapie and transmissible mink encephalopathy in hamsters. II. Clinical signs, pathology and pathogenesis
37. Prion protein conformation in a patient with sporadic fatal insomnia
38. Scrapie prion rod formation in vitro requires both detergent extraction and limited proteolysis
39. Fatal familial insomnia and familial Creutzfeldt-Jakob disease: Different prion proteins determined by a DNA polymorphism
40. Properties of the scrapie prion protein: Quantitative analysis of protease resistance
41. Conversion of α-helices into β-sheets features in the formation of the scrapie prion proteins
42. Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease
43. Scrapie produced experimentally in goats with special reference to the clinical syndrome
44. A conformational transition at the N terminus of the prion protein features in formation of the scrapie isoform
45. Semipreparative chromatographic method to purify the normal cellular isoform of the prion protein in non-denatured form
46. Measurement of the scrapie agent using an incubation time interval assay
47. Scrapie prions aggregate to form amyloid-like birefringent rods
48. Purification and structural studies of a major scrapie prion protein
49. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication
50. Attempts to restore scrapie prion infectivity after exposure to protein denaturants
51. Prion protein biology
52. Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication
53. Data analysis and quality control of assays: A practical primer
54. Epitope mapping of the Syrian hamster prion protein utilizing chimeric and mutant genes in a vaccinia virus expression system
55. Thermal-stability and conformational transitions of scrapie amyloid (prion) protein correlate with infectivity
56. Sc molecules with different conformations
57. Propagation of prion strains through specific conformers of the prion protein
58. Rapid detection of Creutzfeldt-Jakob disease and scrapie prion proteins
59. Structural analysis of the scrapie prion protein using mass spectrometry and amino acid sequencing
60. Regional mapping of prion proteins in brains
61. Prion propagation in mice ex-pressing human and chimeric PrP transgenes implicates the interaction of cellular PrP with another protein
62. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity
63. Separation of scrapie prion infectivity from PrP amyloid polymers
64. Human monoclonal antibodies against a plethora of viral pathogens from single combinatorial libraries
65. Circumventing tolerance to generate autologous monoclonal antibodies to the prion protein
66. Mapping the prion protein using recombinant antibodies
67. Experimental transmission of mouse passaged scrapie to goats, sheep, rats and hamsters
68. Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein