Prions and neurodegenerative diseases

Current Opinion in Genetics and Development

Volumn 10, Issue 5, 2000, Pages 568-574

  Hope, James ^a  

^a INSTITUTE FOR ANIMAL HEALTH   (United Kingdom)

Author keywords

[No Author keywords available]

Indexed keywords

PRION PROTEIN;

DEGENERATIVE DISEASE; GENE EXPRESSION; GENE STRUCTURE; GENETIC ANALYSIS; HUMAN; NONHUMAN; PATHOGENESIS; PRION; PRION DISEASE; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN MODIFICATION; REVIEW; TRANSGENE;

EID: 0033867608     PISSN: 0959437X     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0959-437X(00)00129-5     Document Type: Review

References (62)

1. Prions
2. Prions in Saccharomyces and Podospora spp.: Protein-based inheritance
3. Analysis of 27 mammalian and 9 avian PrPs reveals high conservation of flexible regions of the prion protein
4. Copper binding to the prion protein: Structural implications of four identical cooperative binding sites
5. Influence of amino acid substitutions related to inherited human prion diseases on the thermodynamic stability of the cellular prion protein
6. Expression of amino-terminally truncated PrP in the mouse leading to ataxia and specific cerebellar lesions
7. Prion protein of 106 residues creates an artificial transmission barrier for prion replication in transgenic mice
8. The major polypeptide of scrapie-associated fibrils (Saf) has the same size, charge-distribution and N-terminal protein-sequence as predicted for the normal brain protein (PrP)
9. Ultrastructural immuno-localization of synthetic prion protein peptide antibodies in 87v murine scrapie
10. Dominant-negative inhibition of prion formation diminished by deletion mutagenesis of the prion protein
11. Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects
12. Molecular analysis of ovine prion protein identifies similarities between BSE and an experimental isolate of natural scrapie, CH1641
13. Similar signature of the prion protein in natural sheep scrapie and bovine spongiform encephalopathy-linked diseases
14. Eight prion strains have PrPSc molecules with different conformations
15. PrP (prion) gene expression in sheep may be modulated by alternative polyadenylation of its messenger RNA
16. The prion protein gene - A role in mouse embryogenesis
17. PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain
18. Ectopic expression of prion protein (PrP) in T lymphocytes or hepatocytes of PrP knockout mice is insufficient to sustain prion replication
19. Identification of the end stage of scrapie using infected neural grafts
20. Impaired motor coordination in mice lacking prion protein
21. Ataxia in prion protein (PrP)-deficient mice is associated with upregulation of the novel PrP-like protein Doppel
22. PrP's double causes trouble
23. A mouse prion protein transgene rescues mice deficient for the prion protein gene from Purkinje cell degeneration and demyelination
24. Regulation of protein topology by trans-acting factors at the endoplasmic reticulum
25. Transmissible and genetic prion diseases share a common pathway of neurodegeneration
26. Scrapie-induced neuron loss is reduced by treatment with basic fibroblast growth factor
27. Synapse loss associated with abnormal PrP precedes neuronal degeneration in the scrapie-infected murine hippocampus
28. Detection of apoptosis in murine scrapie
29. Neuronal apoptosis in Creutzfeldt-Jakob disease
30. Astrocytic glutamate uptake and prion protein expression
31. Prion protein peptide neurotoxicity can be mediated by astrocytes
32. Prion protein expression aids cellular uptake and veratridine-induced release of copper
33. Normal prion protein has an activity like that of superoxide dismutase
34. Prion protein peptides: Optimal toxicity and peptide blockade of toxicity
35. Consequences of manganese replacement of copper for the prion protein function and proteinase resistance
36. Prophylactic potential of pentosan polysulphate in transmissible spongiform encephalopathies
37. Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence
38. Novel therapeutic uses for porphyrins and phthalocyanines in the transmissible spongiform encephalopathies - Commentary
39. Screening Congo Red and its analogues for their ability to prevent the formation of PrP-res in scrapie-infected cells
40. Elimination of prions by branched polyamines and implications for therapeutics
41. Reversion of prion protein conformational changes by synthetic beta-sheet breaker peptides
42. Activation of microglial cells by PrP and β-amyloid fragments raises intracellular calcium through L-type voltage sensitive calcium channels
43. Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity
44. Prions prevent neuronal cell-line death
45. The Bcl-2 protein family: Arbiters of cell survival
46. Cultured cell sublines highly susceptible to prion infection
47. Programmed cell death and Bcl-2 protection in very low oxygen
48. Degeneration of skeletal-muscle, peripheral-nerves, and the central-nervous-system in transgenic mice overexpressing wild-type prion proteins
49. Accumulation of protease-resistant prion protein (PrP) and apoptosis of cerebellar granule cells in transgenic mice expressing a PrP inserfional mutation
50. A single amino acid alteration (101L) introduced into murine PrP dramatically alters incubation time of transmissible spongiform encephalopathy
51. A synthetic peptide initiates Gerstmann-Straussler-Scheinker (GSS) disease in transgenic mice
52. In vivo toxicity of prion protein in murine scrapie: Ultrastructural and immunogold studies
53. Cytotoxicity of prion protein peptide (Prp105-126) differs in mechanism from the cytotoxic activity of the Alzheimers-Disease amyloid peptide, a-Beta-25-35
54. Apoptosis-mediated neurotoxicity induced by beta-amyloid and Prp fragments
55. Neurotoxicity of prion peptide 106-126 not confirmed
56. Cell death in prion disease
57. Mechanisms of Prion(Sc)- and HIV-1 gp120 induced neuronal cell death
58. 2+ in microglia activated by prion protein fragment
59. Molecular determinants of the physicochemical properties of a critical prion protein region comprising residues 106-126
60. A neurotoxic prion protein-fragment enhances proliferation of microglia but not astrocytes in culture
61. Role of microglia and host prion protein in neurotoxicity of a prion protein-fragment
62. Effects of oxidative stress on prion protein expression in PC12 cells