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. |