| http://purl.uniprot.org/citations/17942075 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/17942075 | http://www.w3.org/2000/01/rdf-schema#comment | "Pseudoxanthoma elasticum (PXE), a heritable multi-system disorder manifesting with ectopic mineralization of soft connective tissues, is caused by mutations in the ABCC6/MRP6 gene/protein system, but the mechanisms how the ABCC6 mutations lead to aberrant mineralization are currently unknown. In this study, we utilized a transgenic mouse model, Abcc6-/-, to examine the mineralization processes. We focused on matrix gla protein (MGP) which has been shown to be critical, when activated by gamma-carboxylation of glutamyl residues, for prevention of unwanted mineralization. The concentration of MGP in the serum of Abcc6-/-mice was significantly reduced when compared to wild-type controls (p<0.004). More importantly, MGP isolated from the liver of Abcc6-/-mice was largely under-carboxylated and therefore possesses no activity. Finally, examination of the Abcc6-/-mice revealed association of total and under-carboxylated forms of MGP with ectopic mineralization while the gamma-carboxylated form was essentially absent. These results suggest that MGP in Abcc6-/-mice is largely in inactive form and is unable to prevent the unwanted mineralization of connective tissues in PXE."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.bbrc.2007.09.122"xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/author | "Jiang Q."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/author | "Li Q."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/author | "Uitto J."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/author | "Schurgers L.J."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/date | "2007"xsd:gYear |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/name | "Biochem Biophys Res Commun"xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/pages | "208-213"xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/title | "Pseudoxanthoma elasticum: reduced gamma-glutamyl carboxylation of matrix gla protein in a mouse model (Abcc6-/-)."xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://purl.uniprot.org/core/volume | "364"xsd:string |
| http://purl.uniprot.org/citations/17942075 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/17942075 |
| http://purl.uniprot.org/citations/17942075 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/17942075 |
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| http://purl.uniprot.org/uniprot/#_P19788-mappedCitation-17942075 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/17942075 |
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