부산대학교 도서관

Abstract The endothelin system is a vertebrate-specific innovation with important roles in regulating the cardiovascular system and renal and pulmonary processes, as well as the development of the vertebrate-specific neural crest cell population and its derivatives. This system is comprised of three structurally similar 21-amino acid peptides that bind and activate two G-protein coupled receptors. In 1994, knockouts of the Edn3 and Ednrb genes revealed their crucial function during development of the enteric nervous system and melanocytes, two neural-crest derivatives. Since then, human and mouse genetics, combined with cellular and developmental studies, have helped to unravel the role of this signaling pathway during development and adulthood. In this review, we will summarize the known functions of the EDN3/EDNRB pathway during neural crest development, with a specific focus on recent scientific advances, and the enteric nervous system in normal and pathological conditions. Highlights • The endothelin system has crucial roles in development of the neural crest. • EDN3/EDNRB pathway controls enteric nervous system and melanocyte development. • In human, mutations are found in Waardenburg syndrome and Hirschsprung disease • Similar phenotypic observations are found in animal models. • In ENS, the EDN3/EDNRB pathway mainly controls progenitor's proliferation, differentiation and migration. [ABSTRACT FROM AUTHOR]