Anti-BMPR2: Mouse BMPR2 Antibody

Mutations in BMPR2 are a cause of pulmonary arterial hypertension, a rare autosomal dominant disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. Eventually these lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death.⁴ Mutations in BMPR2 cause a loss of function or dominant negative effect and can be found in the extracellular ligand-binding domain, in the kinase domain, or in the long cytoplasmic tail, all of which can affect the signaling mechanism as well as interaction of the receptor with the cytoskeleton. The disease phenotype can be mimicked in heterozygous mutant mice which develop increased arterial pressure and pulmonary vascular resistance compared with their wild-type littermates. However, homozygous knockout mice die at the gastrulation stage of embryogenesis consistent with a critical role for BMPR2 in development. Loss of BMPR2 is also associated with some cancer types and is linked to a 5-year survival rate in human prostate cancer.⁵ Overexpression of a dominant negative form of BMPR2 has been shown to inhibit growth of human breast cancer cells. Finally, in mice conditional inactivation of BMPR2 in the stroma leads to epithelial hyperplasia throughout the colon with increased epithelial cell proliferation and polyp formation.