The functional links between prion protein and copper

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-...

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Autores principales:	VARELA-NALLAR,LORENA, TOLEDO,ENRIQUE M, CHACÓN,MARCELO A, INESTROSA,NIBALDO C
Lenguaje:	English
Publicado:	Sociedad de Biología de Chile 2006
Materias:	Prion protein copper heparin HSPG Prnp MRE
Acceso en línea:	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602006000100005
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spelling	oai:scielo:S0716-976020060001000052006-06-30The functional links between prion protein and copperVARELA-NALLAR,LORENATOLEDO,ENRIQUE MCHACÓN,MARCELO AINESTROSA,NIBALDO C Prion protein copper heparin HSPG Prnp MRE 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.info:eu-repo/semantics/openAccessSociedad de Biología de ChileBiological Research v.39 n.1 20062006-01-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602006000100005en10.4067/S0716-97602006000100005
institution	Scielo Chile
collection	Scielo Chile
language	English
topic	Prion protein copper heparin HSPG Prnp MRE
spellingShingle	Prion protein copper heparin HSPG Prnp MRE VARELA-NALLAR,LORENA TOLEDO,ENRIQUE M CHACÓN,MARCELO A INESTROSA,NIBALDO C The functional links between prion protein and copper
description	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.
author	VARELA-NALLAR,LORENA TOLEDO,ENRIQUE M CHACÓN,MARCELO A INESTROSA,NIBALDO C
author_facet	VARELA-NALLAR,LORENA TOLEDO,ENRIQUE M CHACÓN,MARCELO A INESTROSA,NIBALDO C
author_sort	VARELA-NALLAR,LORENA
title	The functional links between prion protein and copper
title_short	The functional links between prion protein and copper
title_full	The functional links between prion protein and copper
title_fullStr	The functional links between prion protein and copper
title_full_unstemmed	The functional links between prion protein and copper
title_sort	functional links between prion protein and copper
publisher	Sociedad de Biología de Chile
publishDate	2006
url	http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602006000100005
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The functional links between prion protein and copper

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