Prion diseases, metals and antioxidants

P Davies, D R Brown

Research output: Chapter in Book/Report/Conference proceedingChapter

  • 1 Citations

Abstract

Prion diseases are rare neurodegenerative diseases but have gained considerable notoriety because of BSE and its links to the human disease, variant CJD. However, prion diseases, like other amyloidogenic diseases, are linked to changes to a key protein. The prion protein's change in conformation is considered the pivotal event that leads to prion disease. However, this mysterious protein has been hard to understand in terms of its role in the cell and the events that trigger this conformational change. One of the key findings that have changed the way we view these diseases is discovery of the ability of the prion protein to bind metals. In particular the normal isoform of the prion protein binds copper. Expression of the prion protein by cells has also been shown to protect cells, particularly from oxidative damage. Binding of the "wrong" metal, manganese plays a role in protein conversion. Therefore the study of metals in prion disease has proven a very rich ground for understanding these complex diseases and the enigmatic protein associated with them, the prion protein. This chapter provides an up-do-date review of the current knowledge of the prion protein as a metalloprotein.
LanguageEnglish
Title of host publicationBrain Diseases and Metalloproteins
EditorsD R Brown
Place of PublicationSingapore
PublisherPan Stanford Publishing
Pages249-293
Number of pages45
ISBN (Print)9789814316019
DOIs
StatusPublished - 31 Jul 2012

Fingerprint

Prion Diseases
Antioxidants
Metals
Proteins
Metalloproteins
Manganese
Rare Diseases
Neurodegenerative Diseases
Prion Proteins
Copper
Protein Isoforms

Cite this

Davies, P., & Brown, D. R. (2012). Prion diseases, metals and antioxidants. In D. R. Brown (Ed.), Brain Diseases and Metalloproteins (pp. 249-293). Singapore: Pan Stanford Publishing. https://doi.org/10.4032/9789814364072

Prion diseases, metals and antioxidants. / Davies, P; Brown, D R.

Brain Diseases and Metalloproteins . ed. / D R Brown. Singapore : Pan Stanford Publishing, 2012. p. 249-293.

Research output: Chapter in Book/Report/Conference proceedingChapter

Davies, P & Brown, DR 2012, Prion diseases, metals and antioxidants. in DR Brown (ed.), Brain Diseases and Metalloproteins . Pan Stanford Publishing, Singapore, pp. 249-293. https://doi.org/10.4032/9789814364072
Davies P, Brown DR. Prion diseases, metals and antioxidants. In Brown DR, editor, Brain Diseases and Metalloproteins . Singapore: Pan Stanford Publishing. 2012. p. 249-293 https://doi.org/10.4032/9789814364072
Davies, P ; Brown, D R. / Prion diseases, metals and antioxidants. Brain Diseases and Metalloproteins . editor / D R Brown. Singapore : Pan Stanford Publishing, 2012. pp. 249-293
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