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http://purl.uniprot.org/citations/9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/9449665http://www.w3.org/2000/01/rdf-schema#comment"Recent gene targeting studies have revealed unexpected roles for endothelins in the development of neural crest-derived tissues. Endothelin converting enzyme-1 (ECE-1) catalyzes the proteolytic activation of big endothelin-1 to endothelin-1(ET-1) in vitro. However, the importance of ECE-1 cleavage in the multiple endothelin pathways in vivo is unknown. Here we generated a targeted null mutation in the mouse ECE-1 gene. ECE-1-/-term embryos exhibited craniofacial and cardiac abnormalities virtually identical to the defects seen in ET-1 and endothelin A receptor (ETA)-deficient embryos. Epidermal melanocytes as well as enteric neurons of the distal gut were also absent in ECE-1-/-embryos, reproducing the developmental phenotype seen in ET-3-/- and endothelin B receptor (ETB)-/-mice. Surprisingly, large amounts of mature ET-1 peptide are found in ECE-1-/-embryos, indicating that non-ECE-1 protease(s) can activate ET-1 at certain sites. However, these enzymes cannot produce sufficient mature endothelin at the locations crucial for normal embryonic development. These findings reveal that ECE-1 is a bona fide activating protease for both big ET-1 and big ET-3 in vivo, and that the cell-cell communication pathways represented by the ET-1/ECE-1/ETA axis and the ET-3/ECE-1/ETB axis are each involved in the development of distinct subsets of neural crest cell lineages. Mutations in ECE-1 may cause developmental defects in humans, such as Hirschsprung disease, velocardiofacial syndrome and related neurocristopathies."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.org/dc/terms/identifier"doi:10.1242/dev.125.5.825"xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Yanagisawa M."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Hammer R.E."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Richardson J.A."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Emoto N."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Yanagisawa H."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Williams S.C."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Clouthier D.E."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"Kapur R.P."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/author"de Wit D."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/date"1998"xsd:gYear
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/name"Development"xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/pages"825-836"xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/title"Dual genetic pathways of endothelin-mediated intercellular signaling revealed by targeted disruption of endothelin converting enzyme-1 gene."xsd:string
http://purl.uniprot.org/citations/9449665http://purl.uniprot.org/core/volume"125"xsd:string
http://purl.uniprot.org/citations/9449665http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/9449665
http://purl.uniprot.org/citations/9449665http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/9449665
http://purl.uniprot.org/uniprot/#_E0CZB7-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665
http://purl.uniprot.org/uniprot/#_P15331-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665
http://purl.uniprot.org/uniprot/#_A0A2R8VKR9-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665
http://purl.uniprot.org/uniprot/#_A0A2R8W6R6-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665
http://purl.uniprot.org/uniprot/#_A0A183ZRM3-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665
http://purl.uniprot.org/uniprot/#_B1AXF8-mappedCitation-9449665http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/9449665