Activin receptor type-2B (ACTR-IIB), upon ligand binding, forms a receptor comple, Xenopus/Amphibian, consisting of two type II and two type I transmembrane serine/threonine kinases. Type II receptors phosphorylate and activate type I receptors which autophosphorylate, then bind and activate SMAD transcriptional regulators. Defects in ACTR-IIB are the cause of visceral heterota, Xenopus/Amphibian,y autosomal type 4 (HT, Xenopus/Amphibian,4), a form of visceral heterota, Xenopus/Amphibian,y, a comple, Xenopus/Amphibian, disorder due to disruption of the normal left-right asymmetry of the thoracoabdominal organs. HT, Xenopus/Amphibian,4 results in an abnormal arrangement of visceral organs and a wide variety of congenital defects.