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The cellular prion protein binds copper in vivo

, : The cellular prion protein binds copper in vivo. Nature 390(6661): 684-687

The normal cellular form of prion protein (PrPC) is a precursor to the pathogenic protease-resistant forms (PrPSc) believed to cause scrapie, bovine spongiform encephalopathy (BSE) and Creutzfeldt-Jakob disease. Its amino terminus contains the octapeptide PHGGGWGQ, which is repeated four times and is among the best preserved regions of mammalian PrPC. Here we show that the amino-terminal domain of PrPC exhibits five to six sites that bind copper (CU(II)) presented as a glycine chelate. At neutral pH, binding occurs with positive cooperativity, with binding affinity compatible with estimates for extracellular, Labile copper. Two lines of independently derived PrPC gene-ablated (Prnp0/0) mice exhibit severe reductions in the copper content of membrane-enriched brain extracts and similar reductions in synaptosomal and endosome-enriched subcellular fractions. Prnp0/0 mice also have altered cellular phenotypes, including a reduction in the activity of copper/zinc superoxide dismutase and altered electrophysiological responses in the presence of excess copper. These findings indicate that PrPC can exist in a Cu-metalloprotein form in vivo.

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Accession: 009547553

PMID: 9414160

DOI: 10.1038/37783

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Kuszak, J.; Maisel, H.; Harding, C. V., 1978: Gap junctions of chick lens fiber cells. Cellular gap junctions of chick lens fibers are described using scanning electron microscopy and freeze fracture techniques. A large number of cell surface plaques are found. Plaques are implicated in exchange of metabolites between the lens and a...

Pernille Gerstenberg Kirkeby, 2011: Innovations in margarines, shortenings and spreads. Product trends within the oils and fats segment are reviewed with special focus on two case studies which cover the production of low fat dairy spreads compounded from concentrated cream and vegetable oil, and the manufacturing of fat flakes based...

Muhlfeld, Clint C.; Giersch, J. Joseph; Hauer, F. Richard; Pederson, Gregory T.; Luikart, Gordon; Peterson, Douglas P.; Downs, Christopher C.; Fagre, Daniel B., 2011: Climate change links fate of glaciers and an endemic alpine invertebrate; a letter. Climate warming in the mid- to high-latitudes and high-elevation mountainous regions is occurring more rapidly than anywhere else on Earth, causing extensive loss of glaciers and snowpack. However, little is known about the effects of climate chan...

Fior, R.; Vita, N.; Raphael, M.; Minty, A.; Maillot, M.C.; Crevon, M.C.; Caput, D.; Biberfeld, P.; Ferrara, P.; Galanaud, P., 1994: Interleukin-13 gene expression by malignant and EBV-transformed human B lymphocytes. Expression of the IL-13 gene in malignant tissues from 26 human B-cell lymphoid malignancies was analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). A positive signal was detected in 16 cases, which included high grade B lymphoma...

Larionov, Yu M.; Broitman, V. I.; Kouryshkin, V. I., 1999: Hygienic peculiarities of work conditions in trials of heavy agricultural machines. Meditsina Truda i Promyshlennaya Ekologiya 0(10): 32-34

Perrone, J. A., 1996: Generating acceleration sensitive motion sensors from sets of spatio-temporal filters. Investigative Ophthalmology & Visual Science 37(3): S750

Vaisman, A. I.; El'garov, A. A., 1996: Highway medicine Experience, unresolved problems. Meditsina Truda i Promyshlennaya Ekologiya 0(8): 29-31

Jansson, A.; Ronquist, G.; Frithz, G., 1991: Phospholipase A activity in seminal plasma of men attending an infertility clinic. Seminal plasma from 40 men attending an infertility clinic was analyzed for phospholipase A activity and compared with serum from 20 healthy blood donors. The imprecision of the method was acceptable and the overall coefficient of variation was 8....

Potekhina, E. P.; Tyrnov, V. S., 1975: Induced mutagenesis in maize haploids. The effect of treatment with ethyleneimine and ionizing radiation at the haploid level is described and results are presented which indicate the possibility of obtaining homozygous diploid mutant lines from haploids.

Cumings, J. N., 1960: The chemical basis of phenylketonuria. Proc Internatl Med Conf 1: 306-312