{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T14:13:01Z","timestamp":1791295981882,"version":"4.1.0"},"reference-count":60,"publisher":"The Company of Biologists","issue":"5","license":[{"start":{"date-parts":[[1998,3,1]],"date-time":"1998-03-01T00:00:00Z","timestamp":888710400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.biologists.com\/user-licence-1-1\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[1998,3,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Recent gene targeting studies have revealed unexpected roles for endothelins in the development of neural crestderived 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\u2212\/\u2212 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\u2212\/\u2212 embryos, reproducing the developmental phenotype seen in ET-3\u2212\/\u2212 and endothelin B receptor (ETB)\u2212\/\u2212 mice. Surprisingly, large amounts of mature ET-1 peptide are found in ECE-1\u2212\/\u2212 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.<\/jats:p>","DOI":"10.1242\/dev.125.5.825","type":"journal-article","created":{"date-parts":[[2021,4,26]],"date-time":"2021-04-26T03:33:01Z","timestamp":1619407981000},"page":"825-836","source":"Crossref","is-referenced-by-count":428,"title":["Dual genetic pathways of endothelin-mediated intercellular signaling revealed by targeted disruption of endothelin converting enzyme-1 gene"],"prefix":"10.1242","volume":"125","author":[{"given":"Hiromi","family":"Yanagisawa","sequence":"first","affiliation":[{"name":"2 Department of Biochemistry"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masashi","family":"Yanagisawa","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"3 Department of Molecular Genetics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Raj P.","family":"Kapur","sequence":"additional","affiliation":[{"name":"University of Washington Medical Center, Seattle, Washington 98195, USA 5 Department of Pathology ,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James A.","family":"Richardson","sequence":"additional","affiliation":[{"name":"The University of Texas Southwestern Medical Center at Dallas, 5323 Harry Hines Boulevard, Dallas, Texas 75235-9050, USA 4 Department of Pathology ,"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S. Clay","family":"Williams","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"3 Department of Molecular Genetics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David E.","family":"Clouthier","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"3 Department of Molecular Genetics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Damiane de","family":"Wit","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"3 Department of Molecular Genetics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Noriaki","family":"Emoto","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"3 Department of Molecular Genetics"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert E.","family":"Hammer","sequence":"additional","affiliation":[{"name":"1 Howard Hughes Medical Institute"},{"name":"2 Department of Biochemistry"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[1998,3,1]]},"reference":[{"key":"2025021500504661400_DEVELOP_125_5_825C1","doi-asserted-by":"publisher","first-page":"730","DOI":"10.1038\/348730a0","article-title":"Cloning and expression of a cDNA encoding an endothelin receptor","volume":"348","author":"Arai","year":"1990","journal-title":"Nature"},{"key":"2025021500504661400_DEVELOP_125_5_825C2","doi-asserted-by":"publisher","first-page":"1277","DOI":"10.1016\/0092-8674(94)90018-3","article-title":"Interaction of endothelin-3 with endothelin-B receptor is essential for development of epidermal melanocytes and enteric neurons","volume":"79","author":"Baynash","year":"1994","journal-title":"Cell"},{"key":"2025021500504661400_DEVELOP_125_5_825C3","doi-asserted-by":"publisher","first-page":"75","DOI":"10.1002\/(SICI)1097-0177(199701)208:1&lt;75::AID-AJA7&gt;3.0.CO;2-Z","article-title":"Temporospatial expression of the small HSP\/\u03b1B-crystallin in cardiac and skeletal muscle during mouse development","volume":"208","author":"Benjamin","year":"1997","journal-title":"Dev. 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