TY - JOUR
T1 - Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy
AU - Prusiner, Stanley B.
AU - Füzi, Miklós
AU - Scott, Michael
AU - Serban, Dan
AU - Serban, Hana
AU - Taraboulos, Albert
AU - Gabriel, Jean Marc
AU - Wells, Gerald A.H.
AU - Wilesmith, John W.
AU - Bradley, Raymond
AU - DeArmond, Stephen J.
AU - Kristensson, Krister
PY - 1993/3
Y1 - 1993/3
N2 - Bovine spongiform encephalopathy (BSE) is a transmissible neurodegenerative disease. Six brain regions from 11 cattle were examined for the presence of the abnormal isoform of the prion protein (PrPBSE). The highest concentrations of PrpBSE were found in the brain stem, where the greatest degree of spongiform change was observed. Molecular cloning of the bovine PrP gene showed that it encodes a protein of 256 or 264 amino acids with five or six Gly:Pro-rich octarepeats, respectively, in contrast to all other mammalian PrP genes, which encode only five octarepeats. The bovine PrP gene is single copy, and the entire open-reading frame lies within a single exon. Since the transmission of prions across species seems to be restricted by differencesin PrP sequence, the high degree of homology between sheep and bovine PrP (98%) correlates with the proposed cause of BSE.
AB - Bovine spongiform encephalopathy (BSE) is a transmissible neurodegenerative disease. Six brain regions from 11 cattle were examined for the presence of the abnormal isoform of the prion protein (PrPBSE). The highest concentrations of PrpBSE were found in the brain stem, where the greatest degree of spongiform change was observed. Molecular cloning of the bovine PrP gene showed that it encodes a protein of 256 or 264 amino acids with five or six Gly:Pro-rich octarepeats, respectively, in contrast to all other mammalian PrP genes, which encode only five octarepeats. The bovine PrP gene is single copy, and the entire open-reading frame lies within a single exon. Since the transmission of prions across species seems to be restricted by differencesin PrP sequence, the high degree of homology between sheep and bovine PrP (98%) correlates with the proposed cause of BSE.
UR - http://www.scopus.com/inward/record.url?scp=0027466091&partnerID=8YFLogxK
U2 - 10.1093/infdis/167.3.602
DO - 10.1093/infdis/167.3.602
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C2 - 8440932
AN - SCOPUS:0027466091
SN - 0022-1899
VL - 167
SP - 602
EP - 613
JO - Journal of Infectious Diseases
JF - Journal of Infectious Diseases
IS - 3
ER -