Molecular genetics of transmissible spongiform encephalopathies: An introduction

Journal of Toxicological Sciences

Volumn 27, Issue 2, 2002, Pages 69-77

  Weissmann, Charles ^a  

^a UNIVERSITY COLLEGE LONDON   (United Kingdom)

Author keywords

BSE (Bovine spongiform encephalopathy); CJD (Creutzfeldt Jakob disease); Prion; Prion gene knockout; Protein only hypothesis

Indexed keywords

GENE PRODUCT; AMYLOID; INFECTIOUS AMYLOID PRECURSOR PROTEIN; PRION PROTEIN; PRNP PROTEIN, HUMAN; PRNP PROTEIN, MOUSE; PROTEIN PRECURSOR;

CONTROLLED STUDY; CREUTZFELDT JAKOB DISEASE; GENE DISRUPTION; GENE EXPRESSION; GENETIC SUSCEPTIBILITY; KNOCKOUT MOUSE; MOLECULAR GENETICS; MOUSE; NONHUMAN; PRION DISEASE; REVIEW; ANIMAL; DISEASE MODEL; GENETICS; HUMAN; MOUSE MUTANT; MUTATION; PATHOGENICITY; PRION;

BOVINAE; MURINAE;

AMYLOID; ANIMALS; CREUTZFELDT-JAKOB SYNDROME; DISEASE MODELS, ANIMAL; HUMANS; MICE; MICE, KNOCKOUT; MUTATION; PRION DISEASES; PRIONS; PROTEIN PRECURSORS; PRPC PROTEINS; PRPSC PROTEINS;

EID: 0036015317     PISSN: 03881350     EISSN: 18803989     Source Type: Journal    
DOI: 10.2131/jts.27.69     Document Type: Review

References (67)

1. MS-8209, an amphotericin B analogue, delays the appearance of spongiosis, astrogliosis and PrPres accumulation in the brain of scrapie-infected hamsters
2. Does the agent of scrapie replicate without nucleic acid?
3. Scrapie and cellular PrP isoforms are encoded by the same chromosomal gene
4. Nongenetic propagation of strain-specific properties of scrapie prion protein
5. Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy
6. c) is required for scrapie spread within the central nervous system
7. Prion protein expression aids cellular uptake and veratridine-induced release of copper
8. Normal prion protein has an activity like that of superoxide dismutase
9. The basis of strain variation in scrapie
10. Mice devoid of PrP are resistant to scrapie
11. Normal development and behaviour of mice lacking the neuronal cell-surface PrP protein
12. The scrapie-associated form of PrP is made from a cell surface precursor that is both protease- and phospholipase-sensitive
13. Secondary structure analysis of the scrapie-associated protein PrP 27-30 in water by infrared spectroscopy
14. Neurological illness in transgenic mice expressing a prion protein with an insertional mutation
15. + currents
16. Molecular analysis of prion strain variation and the aetiology of 'new variant' CJD
17. Structural aspects of Congo red as an inhibitor of protease-resistant prion protein formation
18. Scrapie prion protein accumulation by scrapie-infected neuroblastoma cells abrogated by exposure to a prion protein antibody
19. Prion protein (PrP) with amino-proximal deletions restoring susceptibility of PrP knockout mice to scrapie
20. Prion protein devoid of the octapeptide repeat region restores susceptibility to scrapie in PrP knockout mice
21. Genetic analysis of Creutzfeldt-Jakob disease and related disorders
22. Cellular prion protein binds laminin and mediates neuritogenesis
23. Self-replication and scrapie
24. Prevention of scrapie pathogenesis by transgenic expression of anti-prion protein antibodies
25. The same prion strain causes vCJD and BSE
26. Copper binding to the N-terminal tandem repeat region of mammalian and avian prion protein: Structural studies using synthetic peptides
27. Seeding "one-dimensional crystallization" of amyloid: A pathogenic mechanism in Alzheimer's disease and scrapie?
28. Scrapie-infected mice and PrP knockout mice share abnormal localization and activity of neuronal nitric oxide synthase
29. Pathogenesis of mouse scrapie: Evidence for neural spread of infection to the CNS
30. A crucial role for B cells in neuroinvasive scrapie
31. Identification of multiple quantitative trait loci linked to prion disease incubation period in mice
32. PrP gene dosage determines the timing but not the final intensity or distribution of lesions in scrapie pathology
33. B lymphocyte-restricted expression of prion protein does not enable prion replication in prion protein knockout mice
34. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein doppel
35. Signal transduction through prion protein
36. Heritable disorder resembling neuronal storage disease in mice expressing prion protein with deletion of an alpha-helix
37. A cellular gene encodes scrapie PrP 27-30 protein
38. Copper stimulates endocytosis of the prion protein
39. Strain-specified relative conformational stability of the scrapie prion protein
40. Amphotericin B delays the incubation period of scrapie in intracerebrally inoculated hamsters
41. Porphyrin and phthalocyanine antiscrapie compounds
42. Scrapie prions
43. Molecular biology of prion diseases
44. Prions
45. Transgenetic studies implicate interactions between homologous PrP isoforms in scrapie prion replication
46. Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication
47. Molecular assessment of the potential transmissibilities of BSE and scrapie to humans
48. The human 37-kDa laminin receptor precursor interacts with the prion protein in eukaryotic cells
49. NMR characterization of the full-length recombinant murine prion protein, mPrP(23-231)
50. (Sc) molecules with different conformations
51. No propagation of prions in mice devoid of PrP
52. Loss of cerebellar Purkinje cells in aged mice homozygous for a disrupted PrP gene
53. A protease resistant prion protein isoform is present in urine of animals and humans affected with prion diseases
54. Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions
55. c, in cultured cells
56. o/o mice predisposed to Purkinje cell loss
57. Structural studies of the scrapie prion protein using mass spectrometry and amino acid sequencing
58. Prion protein of 106 residues creates an artifical transmission barrier for prion replication in transgenic mice
59. Interactions between wild-type and mutant prion proteins modulate neurodegeneration in transgenic mice
60. Evidence for the conformation of the pathologic isoform of the prion protein enciphering and propagating prion diversity
61. Copper binding to the prion protein: Structural implications of four identical cooperative binding sites
62. Reversed genetics: A new approach to the elucidation of structure-function relationships
63. Molecular genetics of transmissible spongiform encephalopathies
64. Perspectives: Neurobiology. PrP's double causes trouble
65. Prions of yeast as heritable amyloidoses
66. A new variant of Creutzfeldt-Jakob disease in the UK
67. Prion disease: A loss of antioxidant function?