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• W2471949457 abstract "Shortlyafterdiscoveryof ET-1in1988,theentireendothelinsystemwascharacterized.Theendothelinsystem consists of the three peptides ET-1, ET-2 and ET-3, their G-protein-coupled receptors endothelin receptor A and B (ETRA and ETRB) and the two endothelin-converting enzymes (ECE-1 and ECE-2). Genetically modified animal models are an important tool in biomedical research. Here we describe the key findings obtained from genetically modified animal models either over-expressing compounds of the ET system or lacking these compounds (knockout mice). Results from the different transgenic and knockout models disclose that the ET system plays a major role in embryonic development. Two ET system-dependent neural crest-driven developmental pathways become obvious: one of them being an ET-1/ETAR axis, responsible for cardio-renal function and development as well as cranial development; the other seems to be an ET-3/ETBR mediated signalling pathway.Mutationswithinthisaxisareassociatedwithdisruptions in epidermal melanocytes and enteric neurons. These findings led to the discovery of similar findings in humans with Hirschsprung disease. In adult life the ET system is most important in the cardiovascular system and plays a role in fibrotic remodelling of the heart, lung and kidney as well as in the regulationof waterandsaltexcretion." @default.
• W2471949457 created "2016-07-22" @default.
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• W2471949457 date "2009-01-01" @default.
• W2471949457 modified "2023-09-24" @default.
• W2471949457 title "PATHOPHYSIOLOGY OF THEENDOTHELINSYSTEM-LESSONS FROM GENETICALLYMANIPULATEDANIMALMODELS" @default.
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• W2471949457 hasPublicationYear "2009" @default.
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