NR AJRF
AU Prusiner,S.B.; Füzi,M.; Scott,M.R.D.; Serban,D.; Serban,H.; Taraboulos,A.; Gabriel,J.M.; Wells,G.A.H.; Wilesmith,J.W.; Bradley,R.; DeArmond,S.J.; Kristensson,K.
TI Immunologic and molecular biologic studies of prion proteins in bovine spongiform encephalopathy
QU Journal of Infectious Diseases 1993 Mar; 167(3): 602-13
PT journal article
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 differences in PrP sequence, the high degree of homology between sheep and bovine PrP (98%) correlates with the proposed cause of BSE.
IN Die Gehirne von BSE befallener Rinder weisen im Hirnstamm die größte Konzentration der abnormen Isoform des Prionproteins auf. Das klonierte Rindergen kodiert ein Prionprotein mit 256 oder 264 Aminosäuren und 5 oder 6 Glycin und Prolin-reichen Oktapeptiden, während alle anderen bisher klonierten Prionproteinsequenzen nur 5 Oktapeptidsequenzen enthalten. Die Homologie zwischen den Prionproteinsequenzen von Schaf und Rind wird undefiniert mit 98% angegeben. Die gesamte kodierende Rindersequenz liegt auf einem Exon eines Genes, das in nur einer Kopie pro haploidem Genom vorkommt. Die Autoren meinen, dass die Übertragbarkeit der Krankheit von einer Art auf eine andere, von der Homologie ihrer jeweiligen Prionproteine abhängt.
MH Amino Acid Sequence; Animal; Base Sequence; Brain/microbiology/pathology; Cattle; Cloning, Molecular; Comparative Study; DNA, Viral/chemistry; Encephalopathy, Bovine Spongiform/diagnosis/*microbiology/pathology; Female; Immunoblotting; Male; Molecular Sequence Data; Nerve Tissue Proteins/chemistry/*genetics/*immunology; Open Reading Frames; Polymerase Chain Reaction; Prions/chemistry/*genetics/*immunology/metabolism; Sequence Homology, Amino Acid; Sequence Homology, Nucleic Acid; Support, Non-U.S. Gov't; Support, U.S. Gov't, P.H.S.
AD Department of Neurology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco 94143-0518.
SP englisch
PO USA
OR Prion-Krankheiten 6