Metal ions and prion diseases

Current Opinion in Chemical Biology

Volumn 6, Issue 2, 2002, Pages 187-192

  Lehmann, Sylvain ^a  

^a INSTITUTE OF HUMAN GENETICS   (France)

Author keywords

[No Author keywords available]

Indexed keywords

COPPER ION; GLYCOSAMINOGLYCAN; METAL ION; NICKEL; PRION PROTEIN; SUPEROXIDE DISMUTASE; ZINC ION; COPPER; TRACE ELEMENT;

ANTIOXIDANT ACTIVITY; BINDING AFFINITY; CEREBROSPINAL FLUID; CIRCULAR DICHROISM; GENE MUTATION; HUMAN; ION TRANSPORT; METAL BINDING; NONHUMAN; OXIDATION; PATHOPHYSIOLOGY; PRION DISEASE; PROTEIN BINDING; PROTEIN FUNCTION; REVIEW; ANIMAL; BINDING SITE; GENETICS; METABOLISM; PRION;

ANIMALS; BINDING SITES; COPPER; HUMANS; PRION DISEASES; PRIONS; TRACE ELEMENTS;

EID: 0036525729     PISSN: 13675931     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1367-5931(02)00295-8     Document Type: Review

References (52)

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3. Histopathological similarities between scrapie and cuprizone toxicity in mice
4. Clinical and histological recovery from the scrapie-like spongiform encephalopathy produced in mice by feeding them with cuprizone
5. Aberrant metal binding by prion protein in human prion disease
6. Purification and structural studies of a major scrapie prion protein
7. Purification and properties of the cellular prion protein from Syrian hamster brain
8. Copper binding to the N-terminal tandem repeat region of mammalian and avian prion protein: Structural studies using synthetic peptides
9. Copper binding to the prion protein: Structural implications of four identical cooperative binding sites
10. Raman spectroscopic study on the copper(II) binding mode of prion octapeptide and its pH dependence
11. Copper binding to octarepeat peptides of the prion protein monitored by mass spectrometry
12. Prion protein binds copper within the physiological concentration range
13. Location and properties of metal-binding sites on the human prion protein
14. XAFS study of the high-affinity copper-binding site of human PrP(91-231) and its low-resolution structure in solution
15. Mapping Cu(II) binding sites in prion proteins by diethylpyrocarbonate modification and MALDI-TOF mass spectrometric 'footprinting'
16. Electron paramagnetic resonance evidence for binding of Cu(2+) to the C-terminal domain of the murine prion protein
17. 2+ binding sites in the N-terminal domain of the prion protein by EPR and CD spectroscopy
18. The N-terminal tandem repeat region of human prion protein reduces copper: Role of tryptophan residues
19. Copper refolding of prion protein
20. Structure of the recombinant full-length hamster prion protein PrP(29-231): The N terminus is highly flexible
21. The cellular prion protein binds copper in vivo
22. Brain copper content and cuproenzyme activity do not vary with prion protein expression level
23. Prion protein-deficient neurons reveal lower glutathione reductase activity and increased susceptibility to hydrogen peroxide toxicity
24. Differential contribution of superoxide dismutase activity by prion protein in vivo
25. Antioxidant activity related to copper binding of native prion protein
26. Effects of copper on survival of prion protein knockout neurons and glia
27. Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity
28. Cleavage of the amino-terminus of the prion protein by reactive oxygen species
29. Copper-catalyzed oxidation of the recombinant SHa(29-231) prion protein
30. Selective oxidation of methionine residues in prion proteins
31. Prion protein expression and superoxide dismutase activity
32. Biosynthesis and cellular processing of the prion protein
33. Normal prion protein has an activity like that of superoxide dismutase
34. Prion and prejudice: Normal protein and the synapse
35. Prion infection impairs the cellular response to oxidative stress
36. Prion disease: A loss of antioxidant function?
37. Scrapie-infected mice and PrP knockout mice share abnormal localization and activity of neuronal nitric oxide synthase
38. Signal transduction through prion protein
39. Ablation of the metal ion-induced endocytosis of the prion protein by disease-associated mutation of the octarepeat region
40. Copper stimulates endocytosis of the prion protein
41. Prion protein selectively binds copper(II) ions
42. Effect of Congo red on wild-type and mutated prion proteins in cultured cells
43. Two mutant prion proteins expressed in cultured cells acquire biochemical properties reminiscent of the scrapie isoform
44. Neurological illness in transgenic mice expressing a prion protein with an insertional mutation
45. Strain-specific prion-protein conformation determined by metal ions
46. Copper binding to the PrP isoforms: A putative marker of their conformation and function
47. Reversibility of scrapie inactivation is enhanced by copper
48. Consequences of manganese replacement of copper for prion protein function and proteinase resistance
49. Ecosystems supporting clusters of sporadic TSEs demonstrate excesses of the radical-generating divalent cation manganese and deficiencies of antioxidant co factors Cu, Se, Fe, Zn. Does a foreign cation substitution at prion protein's Cu domain initiate TSE?
50. Copper(II)-induced conformational changes and protease resistance in recombinant and cellular PrP. Effect of protein age and deamidation
51. Copper converts the cellular prion protein into a protease-resistant species that is distinct from the scrapie isoform
52. Copper and zinc binding modulates the aggregation and neurotoxic properties of the prion peptide PrP106-126