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PMID: 9225743 Published · ppublish English Journal Article Research Support, Non-U.S. Gov't

Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity.

Experimental neurology ·Vol. 146 ·No. 1 ·1997-07-00 ·Pages 104-12

Brown DR, Schulz-Schaeffer WJ, Schmidt B, Kretzschmar HA

Abstract

The cellular function of the prion protein (PrPc), a cell surface glycoprotein expressed in neurones and astrocytes, has not been elucidated. Cell culture experiments reveal that cerebellar cells lacking PrPc are more sensitive to oxidative stress and undergo cell death more readily than wild-type cells. This effect is reversible by treatment with vitamin E. In vivo studies show that the activity of Cu/Zn superoxide dismutase is reduced in Prnp gene-ablated (Prnp0/0) mice. Constitutively high Mn superoxide dismutase activity in these animals may compensate for this loss of responsiveness to oxidative stress. These findings suggest that PrPc may influence the activity of Cu/Zn superoxide dismutase and may be important for cellular resistance to oxidative stress.

MeSH Terms

Amino Acid Sequence Amitrole/pharmacology Animals Cattle Cell Survival/drug effects Cells, Cultured Cerebellum/metabolism Cerebral Cortex/cytology,drug effects,metabolism Ditiocarb/pharmacology Embryo, Mammalian Glutamic Acid/pharmacology Ionomycin/pharmacology Isoenzymes/deficiency,metabolism Mice Mice, Inbred C57BL Mice, Inbred Strains Mice, Knockout Molecular Sequence Data NG-Nitroarginine Methyl Ester/pharmacology Neurons/cytology,drug effects,metabolism Oxidative Stress Peptide Fragments/chemistry,pharmacology Potassium Cyanide/pharmacology PrPC Proteins/genetics,metabolism Prions/chemistry,pharmacology Superoxide Dismutase/deficiency,metabolism Vitamin E/pharmacology

Chemicals

Isoenzymes Peptide Fragments PrPC Proteins Prions Vitamin E Glutamic Acid Ionomycin Ditiocarb Superoxide Dismutase Potassium Cyanide NG-Nitroarginine Methyl Ester Amitrole

Authors & Affiliations

4 authors, click to expand affiliations / ORCID

Brown D R

Institut für Neuropathologie, Universität Göttingen, Germany.

Schulz-Schaeffer W J

Schmidt B

Kretzschmar H A

Article Info

Journal

Experimental neurology

Abbr.

Exp Neurol

ISSN

0014-4886

DOI

10.1006/exnr.1997.6505

Published

1997-07-00

Pages

104-12

Language

English

Region

United States

NLM ID

0370712

Subset

External Links

PubMed source DOI: 10.1006/exnr.1997.6505 View journal details

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Prion protein-deficient cells show altered response to oxidative stress due to decreased SOD-1 activity.

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