| http://purl.uniprot.org/citations/31316208 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/31316208 | http://www.w3.org/2000/01/rdf-schema#comment | "Lamin A/C (LMNA) is one of the most frequently mutated genes associated with dilated cardiomyopathy (DCM). DCM related to mutations in LMNA is a common inherited cardiomyopathy that is associated with systolic dysfunction and cardiac arrhythmias. Here we modelled the LMNA-related DCM in vitro using patient-specific induced pluripotent stem cell-derived cardiomyocytes (iPSC-CMs). Electrophysiological studies showed that the mutant iPSC-CMs displayed aberrant calcium homeostasis that led to arrhythmias at the single-cell level. Mechanistically, we show that the platelet-derived growth factor (PDGF) signalling pathway is activated in mutant iPSC-CMs compared to isogenic control iPSC-CMs. Conversely, pharmacological and molecular inhibition of the PDGF signalling pathway ameliorated the arrhythmic phenotypes of mutant iPSC-CMs in vitro. Taken together, our findings suggest that the activation of the PDGF pathway contributes to the pathogenesis of LMNA-related DCM and point to PDGF receptor-β (PDGFRB) as a potential therapeutic target."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.org/dc/terms/identifier | "doi:10.1038/s41586-019-1406-x"xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Chen X."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Lee J."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Lau E."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Wu H."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Zhang J.Z."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Wu J.C."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Chang H.Y."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Snyder M.P."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Chaudhary R."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Garg P."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Rhee J.W."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Wang P.J."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Diecke S."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Ameen M."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Lam C.K."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Churko J.M."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Karakikes I."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Sallam K."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Seeger T."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Termglinchan V."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Itzhaki I."xsd:string |
| http://purl.uniprot.org/citations/31316208 | http://purl.uniprot.org/core/author | "Greenhaw M."xsd:string |