The EDNRB gene encodes a non-specific, G protein-coupled receptor for endothelin 1, 2, and 3. Once activated through ligand binding, this G protein-coupled receptor known as Endothelin Receptor Type B regulates several biological processes including differentiation of melanocytes, development of enteric nervous system, migration of neural crest cells, vasoconstriction/vasodilation, and sensory perception of pain. Mutations in EDNRB gene are associated with Waardenburg Syndrome Type 4A and Hirschsprung Disease 2.