| http://purl.uniprot.org/citations/33328171 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/33328171 | http://www.w3.org/2000/01/rdf-schema#comment | "Congenital tracheomalacia, resulting from incomplete tracheal cartilage development, is a relatively common birth defect that severely impairs breathing in neonates. Mutations in the Hedgehog (HH) pathway and downstream Gli transcription factors are associated with tracheomalacia in patients and mouse models; however, the underlying molecular mechanisms are unclear. Using multiple HH/Gli mouse mutants including one that mimics Pallister-Hall Syndrome, we show that excessive Gli repressor activity prevents specification of tracheal chondrocytes. Lineage tracing experiments show that Sox9+ chondrocytes arise from HH-responsive splanchnic mesoderm in the fetal foregut that expresses the transcription factor Foxf1. Disrupted HH/Gli signaling results in 1) loss of Foxf1 which in turn is required to support Sox9+ chondrocyte progenitors and 2) a dramatic reduction in Rspo2, a secreted ligand that potentiates Wnt signaling known to be required for chondrogenesis. These results reveal a HH-Foxf1-Rspo2 signaling axis that governs tracheal cartilage development and informs the etiology of tracheomalacia."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.org/dc/terms/identifier | "doi:10.1242/dmm.046573"xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Daniels K."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Wells J.M."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Zorn A.M."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Chaturvedi P."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Kalinichenko V.V."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Nasr T."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Agarwal K."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Sinner D."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Shannon J.M."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Ustiyan V."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Trisno S.L."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Holderbaum A.M."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/author | "Kinney J.L."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/date | "2020"xsd:gYear |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/name | "Dis Model Mech"xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/pages | "dmm.046573"xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://purl.uniprot.org/core/title | "Disruption of a hedgehog-foxf1-rspo2 signaling axis leads to tracheomalacia and a loss of sox9+ tracheal chondrocytes."xsd:string |
| http://purl.uniprot.org/citations/33328171 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/33328171 |
| http://purl.uniprot.org/citations/33328171 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/33328171 |
| http://purl.uniprot.org/uniprot/#_A0A1D5RM76-mappedCitation-33328171 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33328171 |
| http://purl.uniprot.org/uniprot/#_D4Q8H8-mappedCitation-33328171 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33328171 |
| http://purl.uniprot.org/uniprot/#_D4Q8I4-mappedCitation-33328171 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/33328171 |