This gene encodes a member of the bone morphogenetic protein (BMP) receptor family of transmembrane serine/threonine kinases. The ligands of this receptor are BMPs, which are members of the TGF-beta superfamily. BMPs are involved in endochondral bone formation and embryognesis.

These proteins transduce their signals through the formation of hetermeric complexes of two different types of serine (threonine) kinase receptors: type I receptors of about 50-55kD and type I receptors require their respective type II of about 70-80 kD. Type II receptors bind ligands in the absence of type I receptors, but they require their respective type II receptors for ligand binding.

Mutations in this gene have been associated with primary pulmonary hypertension, both familial and fenfluramine-associated, and with pulmonary venoocclusive disease.