Background of the antigen
Endothelins are vasoactive proteins derived form the endothelium The active form of this protein is a 21aa peptide processed from the precursor. The active peptide binds endothelin receptor type B (EDNRB). This interaction EDNRB regulates the development of neural crest-derived cell lineages, such as melanocytes and enteric neurons. Mutations in Endothelin 3 and EDNRB have been associated with Hirschsprung disease (HSCR) and Waardenburg syndrome (WS), which are congenital disorders involving neural crest-derived cells.
