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Publication : Effects of copper on survival of prion protein knockout neurons and glia.

First Author  Brown DR Year  1998
Journal  J Neurochem Volume  70
Issue  4 Pages  1686-93
PubMed ID  9523587 Mgi Jnum  J:46891
Mgi Id  MGI:1202201 Doi  10.1046/j.1471-4159.1998.70041686.x
Citation  Brown DR, et al. (1998) Effects of copper on survival of prion protein knockout neurons and glia. J Neurochem 70(4):1686-93
abstractText  The N-terminal region of the prion protein (PrP) contains an octameric repeat region suggested to bind copper. A 32- amino acid peptide (PrPOcta) based on this region in the protein was tested for its effects on cultured cerebellar cells, Cerebellar cells from mice deficient in cellular PrP (Prnp(0/0) mice) are more sensitive to copper toxicity and oxidative stress. PrPOcta selectively promotes the survival of Prnp(0/0) cerebellar cells, However, PrPOcta also reduces the toxicity of CuSO4 on cerebellar cells and abolishes the difference in increased sensitivity of Prnp(0/0) cells to both copper toxicity and also oxidative stress from xanthine oxidase. PrPOcta does not promote the survival or proliferation of astrocytes or microglia. The survival-promoting effects of PrPOcta on neurons may be due to its ability to effectively chelate copper, The octameric repeat region of PrP may represent a functional domain of the native protein.
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