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Figure 2 Shown from left to right are exons 1–3 in the extracellular domain, involved in dimerization with BMPR1 upon activation by bone morphogenetic protein or other transforming growth factor-beta ligand. Exon 4 spans the transmembrane, and exons 5-11 represent the kinase domain that phosphorylates SMAD proteins. The cytoplasmic tail (exons 12-13) is an unusual feature of transforming growth factor-beta receptors. Most mutations are frameshift or nonsense changes that predict truncation of the gene transcript. PPH = primary pulmonary hypertension.
