dc.date.accessioned | 2014-04-10T16:33:16Z | |
dc.date.available | 2014-04-10T16:33:16Z | |
dc.date.created | 2014-03-12T13:45:12Z | |
dc.date.issued | 2014 | |
dc.identifier.citation | Kong, Xiang Yi Nesset, Cecilie Kåsi Damme, Markus Løberg, Else Marit Lübke, Torben Mæhlen, Jan Andersson, Kristin Brevik Lorenzo, Petra Isabel O. Roos, Norbert Thoresen, G. Hege Rustan, Arild Kase, Eili Tranheim Eskild, Winnie . Loss of lysosomal membrane protein NCU-G1 in mice results in spontaneous liver fibrosis with accumulation of lipofuscin and iron in Kupffer cells. Disease Models and Mechanisms. 2014, 7(3), 351-362 | |
dc.identifier.uri | http://hdl.handle.net/10852/39078 | |
dc.description.abstract | Human kidney predominant protein, NCU-G1, is a highly conserved protein with an unknown biological function. Initially described as a nuclear protein, it was later shown to be a bona fide lysosomal integral membrane protein. To gain insight into the physiological function of NCU-G1, mice with no detectable expression of this gene were created using a gene-trap strategy, and Ncu-g1gt/gt mice were successfully characterized. Lysosomal disorders are mainly caused by lack of or malfunctioning of proteins in the endosomal-lysosomal pathway. The clinical symptoms vary, but often include liver dysfunction. Persistent liver damage activates fibrogenesis and, if unremedied, eventually leads to liver fibrosis/cirrhosis and death. We demonstrate that the disruption of Ncu-g1 results in spontaneous liver fibrosis in mice as the predominant phenotype. Evidence for an increased rate of hepatic cell death, oxidative stress and active fibrogenesis were detected in Ncu-g1gt/gt liver. In addition to collagen deposition, microscopic examination of liver sections revealed accumulation of autofluorescent lipofuscin and iron in Ncu-g1gt/gt Kupffer cells. Because only a few transgenic mouse models have been identified with chronic liver injury and spontaneous liver fibrosis development, we propose that the Ncu-g1gt/gt mouse could be a valuable new tool in the development of novel treatments for the attenuation of fibrosis due to chronic liver damage. | en_US |
dc.language | EN | |
dc.language.iso | en | en_US |
dc.rights | Attribution 3.0 Unported | |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/ | |
dc.title | Loss of lysosomal membrane protein NCU-G1 in mice results in spontaneous liver fibrosis with accumulation of lipofuscin and iron in Kupffer cells | en_US |
dc.type | Journal article | en_US |
dc.creator.author | Kong, Xiang Yi | |
dc.creator.author | Nesset, Cecilie Kåsi | |
dc.creator.author | Damme, Markus | |
dc.creator.author | Løberg, Else Marit | |
dc.creator.author | Lübke, Torben | |
dc.creator.author | Mæhlen, Jan | |
dc.creator.author | Andersson, Kristin Brevik | |
dc.creator.author | Lorenzo, Petra Isabel O. | |
dc.creator.author | Roos, Norbert | |
dc.creator.author | Thoresen, G. Hege | |
dc.creator.author | Rustan, Arild | |
dc.creator.author | Kase, Eili Tranheim | |
dc.creator.author | Eskild, Winnie | |
cristin.unitcode | 185,15,0,0 | |
cristin.unitname | Det matematisk-naturvitenskapelige fakultet | |
cristin.ispublished | true | |
cristin.fulltext | original | |
cristin.qualitycode | 1 | |
dc.identifier.cristin | 1121707 | |
dc.identifier.bibliographiccitation | info:ofi/fmt:kev:mtx:ctx&ctx_ver=Z39.88-2004&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.jtitle=Disease Models and Mechanisms&rft.volume=7&rft.spage=351&rft.date=2014 | |
dc.identifier.jtitle | Disease Models and Mechanisms | |
dc.identifier.volume | 7 | |
dc.identifier.issue | 3 | |
dc.identifier.startpage | 351 | |
dc.identifier.endpage | 362 | |
dc.identifier.doi | http://dx.doi.org/10.1242/dmm.014050 | |
dc.identifier.urn | URN:NBN:no-43945 | |
dc.type.document | Tidsskriftartikkel | en_US |
dc.type.peerreviewed | Peer reviewed | |
dc.source.issn | 1754-8403 | |
dc.identifier.fulltext | Fulltext https://www.duo.uio.no/bitstream/handle/10852/39078/1/Dis.+Model.+Mech.-2014-Kong-351-62.pdf | |
dc.type.version | PublishedVersion | |