http://rdf.ncbi.nlm.nih.gov/pubchem/patent/US-2015361401-A1
Outgoing Links
| Predicate | Object |
|---|---|
| assignee | http://rdf.ncbi.nlm.nih.gov/pubchem/patentassignee/MD5_cbffa3b81b3b862dc7220b5648f5f745 |
| classificationCPCAdditional | http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N2501-606 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N2501-603 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N2501-604 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N2501-602 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N2506-1307 |
| classificationCPCInventive | http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12Q1-68 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/G01N33-5064 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N5-0696 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/G01N33-15 http://rdf.ncbi.nlm.nih.gov/pubchem/patentcpc/C12N5-0607 |
| classificationIPCInventive | http://rdf.ncbi.nlm.nih.gov/pubchem/patentipc/G01N33-50 http://rdf.ncbi.nlm.nih.gov/pubchem/patentipc/C12N5-074 |
| filingDate | 2015-08-28-04:00^^<http://www.w3.org/2001/XMLSchema#date> |
| inventor | http://rdf.ncbi.nlm.nih.gov/pubchem/patentinventor/MD5_e4875f315414b5ee439fcf485f30996f http://rdf.ncbi.nlm.nih.gov/pubchem/patentinventor/MD5_e3e5ce41afd29aa821e8481201208286 |
| publicationDate | 2015-12-17-04:00^^<http://www.w3.org/2001/XMLSchema#date> |
| publicationNumber | US-2015361401-A1 |
| titleOfInvention | Induced pluripotent stem cell model for fabry disease and use thereof |
| abstract | The present invention relates to an induced pluripotent stem cell model of Fabry disease, a preparation method thereof, and a use of the same for the study of Fabry disease development and for the screening of a therapeutic agent for the disease. Particularly, Fabry disease derived induced pluripotent stem cells (iPSCs), embryoid body (EB), and vascular cells were developed and differentiated from fibroblasts originated from Fabry disease patient, wherein the Fabry disease originated iPSCs displayed significantly reduced expression and activity of GLA protein therein, compared with the normal cells, resulting in the accumulation of globotriaosylceramide (Gb3, CD77). Also, the differentiation of vascular cells was induced from the Fabry disease originated iPSCs, and as a result the iPSCs were successfully differentiated into vascular endothelial cells and vascular smooth muscle cells with significantly expressing the marker protein. When the vascular endothelial cells and vascular smooth muscle cells were treated with Fabrazyme, the accumulation of Gb3 was significantly reduced, suggesting that the said cell model can be effectively used for the analysis/study of Fabry disease outbreak mechanism and for the screening of a therapeutic agent for the disease. |
| isCitedBy | http://rdf.ncbi.nlm.nih.gov/pubchem/patent/US-10767164-B2 |
| priorityDate | 2013-12-23-04:00^^<http://www.w3.org/2001/XMLSchema#date> |
| type | http://data.epo.org/linked-data/def/patent/Publication |
Incoming Links
Total number of triples: 357.