| http://purl.uniprot.org/citations/20961863 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/20961863 | http://www.w3.org/2000/01/rdf-schema#comment | "Fabry disease is a lysosomal storage disorder caused by an α-galactosidase A (α-Gal A) deficiency and resulting in the accumulation of glycosphingolipids, predominantly globotriaosylceramide (Gb3). A transgenic mouse expressing the human α-Gal A R301Q mutant in an α-Gal A-knockout background (TgM/KO) should be useful for studying active-site-specific chaperone (ASSC) therapy for Fabry disease. However, the Gb3 content in the heart tissue of this mouse was too low to detect an ASSC-induced effect. To increase the Gb3 levels in mouse organs, we created transgenic mice (TgG3S) expressing human α1,4-galactosyltransferase (Gb3 synthase). High levels of Gb3 were observed in all major organs of the TgG3S mouse. A TgG3S (+/-)M(+/-)/KO mouse was prepared by cross-breeding the TgG3S and TgM/KO mice and the Gb3 content in the heart of the TgG3S(+/-)M(+/-)/KO mouse was 1.4 µg/mg protein, higher than in the TgM(+/-)/KO (<0.1 µg/mg protein). Treatment with an ASSC, 1-deoxygalactonojirimycin, caused a marked induction of α-Gal A activity and a concomitant reduction of the Gb3 content in the TgG3S(+/-) M(+/-)/KO mouse organs. These data indicated that the TgG3S(+/-) M(+/-)/KO mouse was suitable for studying ASSC therapy for Fabry disease, and that the TgG3S mouse would be useful for studying the effect of high Gb3 levels in mouse organs."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.org/dc/terms/identifier | "doi:10.1093/jb/mvq125"xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Ishii S."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Furukawa K."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Mannen K."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Yokoyama S."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Yoshioka H."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Yano S."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Taguchi A."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Noguchi Y."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Kunieda T."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Kulkarni A.B."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Matsuda J."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Uchio-Yamada K."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Hamanaka R."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/author | "Shiozuka C."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/date | "2011"xsd:gYear |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/name | "J Biochem"xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/pages | "161-170"xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/title | "Increased globotriaosylceramide levels in a transgenic mouse expressing human alpha1,4-galactosyltransferase and a mouse model for treating Fabry disease."xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://purl.uniprot.org/core/volume | "149"xsd:string |
| http://purl.uniprot.org/citations/20961863 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/20961863 |
| http://purl.uniprot.org/citations/20961863 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/20961863 |
| http://purl.uniprot.org/uniprot/#_A0A0S2Z671-mappedCitation-20961863 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/20961863 |