dc.contributor.author | Skedsmo, Fredrik Strebel | |
dc.contributor.author | Malachin, Giulia | |
dc.contributor.author | Våge, Dag Inge | |
dc.contributor.author | Hammervold, Mie Marie | |
dc.contributor.author | Salvesen, Øyvind | |
dc.contributor.author | Ersdal, Cecilie | |
dc.contributor.author | Ranheim, Birgit | |
dc.contributor.author | Stafsnes, Marit Hallvardsdotter | |
dc.contributor.author | Bartosova, Zdenka | |
dc.contributor.author | Bruheim, Per | |
dc.contributor.author | Jäderlund, Karin Hultin | |
dc.contributor.author | Matiasek, Kaspar | |
dc.contributor.author | Espenes, Arild | |
dc.contributor.author | Tranulis, Michael A. | |
dc.date.accessioned | 2020-01-29T12:34:46Z | |
dc.date.available | 2020-01-29T12:34:46Z | |
dc.date.created | 2020-01-14T13:04:22Z | |
dc.date.issued | 2019 | |
dc.identifier.issn | 0892-6638 | |
dc.identifier.uri | http://hdl.handle.net/11250/2638612 | |
dc.description.abstract | Studies in mice with ablation of Prnp, the gene that encodes the cellular prion protein (PrPC), have led to the hypothesis that PrPC is important for peripheral nerve myelin maintenance. Here, we have used a nontransgenic animal model to put this idea to the test; namely, goats that, due to a naturally occurring nonsense mutation, lack PrPC. Teased nerve fiber preparation revealed a demyelinating pathology in goats without PrPC. Affected nerves were invaded by macrophages and T cells and displayed vacuolated fibers, shrunken axons, and onion bulbs. Peripheral nerve lipid composition was similar in young goats with or without PrPC, but markedly different between corresponding groups of adult goats, reflecting the progressive nature of the neuropathy. This is the first report of a subclinical demyelinating polyneuropathy caused by loss of PrPC function in a nontransgenic mammal. | nb_NO |
dc.language.iso | eng | nb_NO |
dc.publisher | Wiley | nb_NO |
dc.rights | Navngivelse-Ikkekommersiell 4.0 Internasjonal | * |
dc.rights.uri | http://creativecommons.org/licenses/by-nc/4.0/deed.no | * |
dc.title | Demyelinating polyneuropathy in goats lacking prion protein | nb_NO |
dc.type | Journal article | nb_NO |
dc.type | Peer reviewed | nb_NO |
dc.description.version | publishedVersion | nb_NO |
dc.source.journal | The FASEB Journal | nb_NO |
dc.identifier.doi | 10.1096/fj.201902588R | |
dc.identifier.cristin | 1772357 | |
dc.description.localcode | © 2019 Norwegian University of Life Sciences. The FASEB Journal published by John Wiley & Sons Ltd on behalf of The FASEB Journal This is an open access article under the terms of the Creative Commons Attribution‐NonCommercial License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited and is not used for commercial purposes | nb_NO |
cristin.unitcode | 194,66,15,0 | |
cristin.unitname | Institutt for bioteknologi og matvitenskap | |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 2 | |