| http://purl.uniprot.org/citations/32151560 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/32151560 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/32151560 | http://www.w3.org/2000/01/rdf-schema#comment | "Non-syndromic mitral valve prolapse (MVP) is the most common heart valve disease affecting 2.4% of the population. Recent studies have identified genetic defects in primary cilia as causative to MVP, although the mechanism of their action is currently unknown. Using a series of gene inactivation approaches, we define a paracrine mechanism by which endocardially-expressed Desert Hedgehog (DHH) activates primary cilia signaling on neighboring valve interstitial cells. High-resolution imaging and functional assays show that DHH de-represses smoothened at the primary cilia, resulting in kinase activation of RAC1 through the RAC1-GEF, TIAM1. Activation of this non-canonical hedgehog pathway stimulates α-smooth actin organization and ECM remodeling. Genetic or pharmacological perturbation of this pathway results in enlarged valves that progress to a myxomatous phenotype, similar to valves seen in MVP patients. These data identify a potential molecular origin for MVP as well as establish a paracrine DHH-primary cilium cross-talk mechanism that is likely applicable across developmental tissue types."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.ydbio.2020.03.003"xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.ydbio.2020.03.003"xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Bian J."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Bian J."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Guo L."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Guo L."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Moore R."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Moore R."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Moore K."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Moore K."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Wang C."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Wang C."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Beck T."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Beck T."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "McDowell C."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "McDowell C."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Glover J."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Glover J."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Lipschutz J.H."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Lipschutz J.H."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Abrol S."xsd:string |
| http://purl.uniprot.org/citations/32151560 | http://purl.uniprot.org/core/author | "Abrol S."xsd:string |