Endothelin receptor B (ETBR), also known as EDNRB, ETB, ETRB, HSCR and HSCR2, is a member of the guanine-binding, regulatory protein-coupled receptor family. Three isoforms of ETBR exist called isoform 1, isoform 2 and δ 3. ETBR is involved in the regulation of sodium excretion and glomular filtration rate (GFR). ETBR plays a role in the normal development of the neural crestderived cell lineages, epidermal melanocytes and enteric neurons. ETBR is expressed in lung, kidney, placenta, skeletal muscle and stem villi vessels. Both of the ET receptors, ETAR and ETBR, are activated by ET1, which results in inhibition of active lens sodium-potassium transport. Activation of the ET receptors also causes an increase in cytoplasmic calcium concentration in cultured lens epithelial cells. ETBR deficiency causes early onset dysfunction of the kidney, characterized by reduced sodium excretion, decreased GFR and slightly elevated blood pressure. Mutations in the gene encoding ETBR produce congenital aganglionic megacolon and pigment abnormalities. The multigenic disorder, Hirschsprung’s disease type 2, is also due to a mutation in the ETBR gene.
Background References
1. Tagashira H et al. UVB Stimulates the Expression of Endothelin B Receptor in Human Melanocytes via a Sequential Activation of the p38/MSK1/CREB/MITF Pathway Which Can Be Interrupted by a French Maritime Pine Bark Extract through a Direct Inactivation of MSK1. PLoS One 10:e0128678 (2015).
2. Hall SM et al. Endothelin receptor expression in idiopathic pulmonary arterial hypertension: effect of bosentan and epoprostenol treatment. Eur Respir J 38:851-60 (2011).
Sequence Similarity
Belongs to the G-protein coupled receptor 1 family. Endothelin receptor subfamily. EDNRB sub-subfamily.
Tissue Specificity
Expressed in placental stem villi vessels, but not in cultured placental villi smooth muscle cells.
Post-translational Modification
Palmitoylation of Cys-402 was confirmed by the palmitoylation of Cys-402 in a deletion mutant lacking both Cys-403 and Cys-405.