The functional links between prion protein and copper

Varela-Nallar, L; Toledo EM; Chacón MA; Inestrosa, NC

Abstract

Prion diseases are fatal neurodegenerative disorders associated with the conversion of the cellular prion protein (PrPC) into a pathologic isoform. Although the physiological function of PrPC remains unknown, evidence relates PrPC to copper metabolism and oxidative stress as suggested by its copper-binding properties in the N-terminal octapeptide repeat region. This region also reduces copper ions in vitro, and this reduction ability is associated with the neuroprotection exerted by the octarepeat region against copper in vivo. In addition, the promoter region of the PrPC gene contains putative metal response elements suggesting it may be regulated by heavy metals. Here we address some of the evidence that support a physiological link between PrPC and copper. Also, in vivo experiments suggesting the physiological relevance of PrPC interaction with heparan sulfate proteoglycans are discussed.

Más información

Título según WOS: The functional links between prion protein and copper
Título según SCIELO: The functional links between prion protein and copper
Título de la Revista: BIOLOGICAL RESEARCH
Volumen: 39
Número: 1
Editorial: Springer Nature
Fecha de publicación: 2006
Página de inicio: 39
Página final: 44
Idioma: English
DOI:

10.4067/S0716-97602006000100005

Notas: ISI, SCIELO