Bmpr2

Summary

Gene Symbol: Bmpr2
Description: bone morphogenetic protein receptor, type II (serine/threonine kinase)
Alias: 2610024H22Rik, AL117858, AW546137, BB189135, BMP-2, BMPR-2, BMPR-II, BMPRII, BRK-3, Gm20272, bone morphogenetic protein receptor type-2, BMP type II receptor, BMP type-2 receptor, bone morphogenic protein receptor, type II (serine/threonine kinase)
Species: mouse
Products:     Bmpr2

Top Publications

  1. Beppu H, Mwizerwa O, Beppu Y, Dattwyler M, Lauwers G, Bloch K, et al. Stromal inactivation of BMPRII leads to colorectal epithelial overgrowth and polyp formation. Oncogene. 2008;27:1063-70 pubmed
    ..We observed that conditional inactivation of the BMP type II receptor (BMPRII) in the stroma leads to epithelial hyperplasia throughout the colon with increased epithelial cell ..
  2. Song Y, Jones J, Beppu H, Keaney J, Loscalzo J, Zhang Y. Increased susceptibility to pulmonary hypertension in heterozygous BMPR2-mutant mice. Circulation. 2005;112:553-62 pubmed
    Bone morphogenetic protein receptor-2 (BMPR2)-heterozygous, mutant (BMPR2(+/-)) mice have a genetic trait similar to that of certain patients with idiopathic pulmonary arterial hypertension (IPAH)...
  3. Alastalo T, Li M, Perez V, Pham D, Sawada H, Wang J, et al. Disruption of PPAR?/?-catenin-mediated regulation of apelin impairs BMP-induced mouse and human pulmonary arterial EC survival. J Clin Invest. 2011;121:3735-46 pubmed publisher
    Reduced bone morphogenetic protein receptor 2 (BMPR2) expression in patients with pulmonary arterial hypertension (PAH) can impair pulmonary arterial EC (PAEC) function. This can adversely affect EC survival and promote SMC proliferation...
  4. Schwappacher R, Weiske J, Heining E, Ezerski V, Marom B, Henis Y, et al. Novel crosstalk to BMP signalling: cGMP-dependent kinase I modulates BMP receptor and Smad activity. EMBO J. 2009;28:1537-50 pubmed publisher
    ..cGKI, a key mediator of vasodilation and hypertension diseases, interacts with and phosphorylates the BMP type II receptor (BMPRII)...
  5. Hong K, Lee Y, Lee E, Park S, Han C, Beppu H, et al. Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension. Circulation. 2008;118:722-30 pubmed publisher
    ..Heterozygous mutations in BMPR2 can be detected in 50% to 70% of patients with familial PAH and 10% to 40% of patients with idiopathic PAH...
  6. Davies R, Holmes A, Deighton J, Long L, Yang X, Barker L, et al. BMP type II receptor deficiency confers resistance to growth inhibition by TGF-? in pulmonary artery smooth muscle cells: role of proinflammatory cytokines. Am J Physiol Lung Cell Mol Physiol. 2012;302:L604-15 pubmed publisher
    ..on PASMC proliferation was characterized in three different models of BMPR-II dysfunction: 1) HPAH PASMCs, 2) Bmpr2(+/-) mouse PASMCs, and 3) control human PASMCs transfected with BMPR-II small interfering RNA...
  7. Beppu H, Kawabata M, Hamamoto T, Chytil A, Minowa O, Noda T, et al. BMP type II receptor is required for gastrulation and early development of mouse embryos. Dev Biol. 2000;221:249-58 pubmed
    ..b>BMP type II receptor (BMPR-II) is a type II serine/threonine kinase receptor, which transduces signals for BMPs through ..
  8. Burton V, Ciuclan L, Holmes A, Rodman D, Walker C, Budd D. Bone morphogenetic protein receptor II regulates pulmonary artery endothelial cell barrier function. Blood. 2011;117:333-41 pubmed publisher
    ..We speculate that this may be a key mechanism involved in the initiation of the disease in heritable PAH that results from defects in BMPR-II expression. ..
  9. Délot E, Bahamonde M, Zhao M, Lyons K. BMP signaling is required for septation of the outflow tract of the mammalian heart. Development. 2003;130:209-20 pubmed
    ..We engineered a knockout mouse that expresses a BMP type II receptor that lacks half of the ligand-binding domain...
  10. Yu P, Beppu H, Kawai N, Li E, Bloch K. Bone morphogenetic protein (BMP) type II receptor deletion reveals BMP ligand-specific gain of signaling in pulmonary artery smooth muscle cells. J Biol Chem. 2005;280:24443-50 pubmed
    Bone morphogenetic protein (BMP) ligands signal by binding the BMP type II receptor (BMPR2) or the activin type II receptors (ActRIIa and ActRIIb) in conjunction with type I receptors to activate SMADs 1, 5, and 8, as well as members of ..

Detail Information

Publications86

  1. Beppu H, Mwizerwa O, Beppu Y, Dattwyler M, Lauwers G, Bloch K, et al. Stromal inactivation of BMPRII leads to colorectal epithelial overgrowth and polyp formation. Oncogene. 2008;27:1063-70 pubmed
    ..We observed that conditional inactivation of the BMP type II receptor (BMPRII) in the stroma leads to epithelial hyperplasia throughout the colon with increased epithelial cell ..
  2. Song Y, Jones J, Beppu H, Keaney J, Loscalzo J, Zhang Y. Increased susceptibility to pulmonary hypertension in heterozygous BMPR2-mutant mice. Circulation. 2005;112:553-62 pubmed
    Bone morphogenetic protein receptor-2 (BMPR2)-heterozygous, mutant (BMPR2(+/-)) mice have a genetic trait similar to that of certain patients with idiopathic pulmonary arterial hypertension (IPAH)...
  3. Alastalo T, Li M, Perez V, Pham D, Sawada H, Wang J, et al. Disruption of PPAR?/?-catenin-mediated regulation of apelin impairs BMP-induced mouse and human pulmonary arterial EC survival. J Clin Invest. 2011;121:3735-46 pubmed publisher
    Reduced bone morphogenetic protein receptor 2 (BMPR2) expression in patients with pulmonary arterial hypertension (PAH) can impair pulmonary arterial EC (PAEC) function. This can adversely affect EC survival and promote SMC proliferation...
  4. Schwappacher R, Weiske J, Heining E, Ezerski V, Marom B, Henis Y, et al. Novel crosstalk to BMP signalling: cGMP-dependent kinase I modulates BMP receptor and Smad activity. EMBO J. 2009;28:1537-50 pubmed publisher
    ..cGKI, a key mediator of vasodilation and hypertension diseases, interacts with and phosphorylates the BMP type II receptor (BMPRII)...
  5. Hong K, Lee Y, Lee E, Park S, Han C, Beppu H, et al. Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension. Circulation. 2008;118:722-30 pubmed publisher
    ..Heterozygous mutations in BMPR2 can be detected in 50% to 70% of patients with familial PAH and 10% to 40% of patients with idiopathic PAH...
  6. Davies R, Holmes A, Deighton J, Long L, Yang X, Barker L, et al. BMP type II receptor deficiency confers resistance to growth inhibition by TGF-? in pulmonary artery smooth muscle cells: role of proinflammatory cytokines. Am J Physiol Lung Cell Mol Physiol. 2012;302:L604-15 pubmed publisher
    ..on PASMC proliferation was characterized in three different models of BMPR-II dysfunction: 1) HPAH PASMCs, 2) Bmpr2(+/-) mouse PASMCs, and 3) control human PASMCs transfected with BMPR-II small interfering RNA...
  7. Beppu H, Kawabata M, Hamamoto T, Chytil A, Minowa O, Noda T, et al. BMP type II receptor is required for gastrulation and early development of mouse embryos. Dev Biol. 2000;221:249-58 pubmed
    ..b>BMP type II receptor (BMPR-II) is a type II serine/threonine kinase receptor, which transduces signals for BMPs through ..
  8. Burton V, Ciuclan L, Holmes A, Rodman D, Walker C, Budd D. Bone morphogenetic protein receptor II regulates pulmonary artery endothelial cell barrier function. Blood. 2011;117:333-41 pubmed publisher
    ..We speculate that this may be a key mechanism involved in the initiation of the disease in heritable PAH that results from defects in BMPR-II expression. ..
  9. Délot E, Bahamonde M, Zhao M, Lyons K. BMP signaling is required for septation of the outflow tract of the mammalian heart. Development. 2003;130:209-20 pubmed
    ..We engineered a knockout mouse that expresses a BMP type II receptor that lacks half of the ligand-binding domain...
  10. Yu P, Beppu H, Kawai N, Li E, Bloch K. Bone morphogenetic protein (BMP) type II receptor deletion reveals BMP ligand-specific gain of signaling in pulmonary artery smooth muscle cells. J Biol Chem. 2005;280:24443-50 pubmed
    Bone morphogenetic protein (BMP) ligands signal by binding the BMP type II receptor (BMPR2) or the activin type II receptors (ActRIIa and ActRIIb) in conjunction with type I receptors to activate SMADs 1, 5, and 8, as well as members of ..
  11. West J, Fagan K, Steudel W, Fouty B, Lane K, Harral J, et al. Pulmonary hypertension in transgenic mice expressing a dominant-negative BMPRII gene in smooth muscle. Circ Res. 2004;94:1109-14 pubmed
    ..The type-II receptor (BMPRII) is required for recognition of all BMPs, and targeted deletion of BMPRII in mice results in fetal lethality ..
  12. de Jesus Perez V, Alastalo T, Wu J, Axelrod J, Cooke J, Amieva M, et al. Bone morphogenetic protein 2 induces pulmonary angiogenesis via Wnt-beta-catenin and Wnt-RhoA-Rac1 pathways. J Cell Biol. 2009;184:83-99 pubmed publisher
    Mutations in bone morphogenetic protein (BMP) receptor II (BMPRII) are associated with pulmonary artery endothelial cell (PAEC) apoptosis and the loss of small vessels seen in idiopathic pulmonary arterial hypertension...
  13. Lee Hoeflich S, Causing C, Podkowa M, Zhao X, Wrana J, Attisano L. Activation of LIMK1 by binding to the BMP receptor, BMPRII, regulates BMP-dependent dendritogenesis. EMBO J. 2004;23:4792-801 pubmed
    ..we show that LIM kinase 1 (LIMK1), a key downstream effector of Rho GTPases, colocalizes with the BMP receptor, BMPRII, in the tips of neurites and binds to BMPRII...
  14. Tada Y, Majka S, Carr M, Harral J, Crona D, Kuriyama T, et al. Molecular effects of loss of BMPR2 signaling in smooth muscle in a transgenic mouse model of PAH. Am J Physiol Lung Cell Mol Physiol. 2007;292:L1556-63 pubmed
    ..IPAH) in human patients is associated with mutations in type 2 receptor for the bone morphogenic protein pathway (BMPR2)...
  15. Wertz J, Bauer P. Caveolin-1 regulates BMPRII localization and signaling in vascular smooth muscle cells. Biochem Biophys Res Commun. 2008;375:557-61 pubmed publisher
    Recent studies demonstrate the interaction of BMPRII and caveolin-1 in various cell types. In this study we test the hypothesis that caveolin-1 interacts with and regulates BMPRII-dependent signaling in vascular smooth muscle cells...
  16. Beppu H, Lei H, Bloch K, Li E. Generation of a floxed allele of the mouse BMP type II receptor gene. Genesis. 2005;41:133-7 pubmed
    ..b>BMP type II receptor (BMPR-II) transduces BMP signals by forming heteromeric complexes with and phosphorylating BMP type I ..
  17. Beppu H, Ichinose F, Kawai N, Jones R, Yu P, Zapol W, et al. BMPR-II heterozygous mice have mild pulmonary hypertension and an impaired pulmonary vascular remodeling response to prolonged hypoxia. Am J Physiol Lung Cell Mol Physiol. 2004;287:L1241-7 pubmed
    ..Our results suggest that, in mice, mutation of one copy of the BMPR-II gene causes pulmonary hypertension but impairs the ability of the pulmonary vasculature to remodel in response to prolonged hypoxic breathing. ..
  18. Sieber C, Kopf J, Hiepen C, Knaus P. Recent advances in BMP receptor signaling. Cytokine Growth Factor Rev. 2009;20:343-55 pubmed publisher
    ..Examples of BMP action during development, but also physiological and pathophysiological conditions in the adult organism are presented. ..
  19. Nasim M, Ogo T, Chowdhury H, Zhao L, Chen C, Rhodes C, et al. BMPR-II deficiency elicits pro-proliferative and anti-apoptotic responses through the activation of TGF?-TAK1-MAPK pathways in PAH. Hum Mol Genet. 2012;21:2548-58 pubmed publisher
    ..Heterozygous mutations in the type II receptor for bone morphogenetic protein (BMPR2) underlie the majority of the inherited and familial forms of PAH...
  20. Miyazono K, Kamiya Y, Morikawa M. Bone morphogenetic protein receptors and signal transduction. J Biochem. 2010;147:35-51 pubmed publisher
    ..The recent development of BMP receptor inhibitors may also prove useful for some clinical diseases induced by hyperactivation of the BMP signalling pathways. ..
  21. Podkowa M, Zhao X, Chow C, Coffey E, Davis R, Attisano L. Microtubule stabilization by bone morphogenetic protein receptor-mediated scaffolding of c-Jun N-terminal kinase promotes dendrite formation. Mol Cell Biol. 2010;30:2241-50 pubmed publisher
    ..regulators of microtubule dynamics, and we show that JNKs associate with the carboxy terminus of the BMP receptor, BMPRII. Activation and binding of JNKs to BMPRII is required for BMP7-induced microtubule stabilization and for BMP7-..
  22. Song Y, Coleman L, Shi J, Beppu H, Sato K, Walsh K, et al. Inflammation, endothelial injury, and persistent pulmonary hypertension in heterozygous BMPR2-mutant mice. Am J Physiol Heart Circ Physiol. 2008;295:H677-90 pubmed publisher
    Heterozygous bone morphogenetic protein receptor-II-knockout (BMPR2(+/-)) mice have a similar genetic trait like that in some idiopathic pulmonary arterial hypertension patients...
  23. Frank D, Lowery J, Anderson L, Brink M, Reese J, De Caestecker M. Increased susceptibility to hypoxic pulmonary hypertension in Bmpr2 mutant mice is associated with endothelial dysfunction in the pulmonary vasculature. Am J Physiol Lung Cell Mol Physiol. 2008;294:L98-109 pubmed
    ..arterial hypertension inherit heterozygous mutations of the type 2 bone morphogenetic protein (BMP) receptor BMPR2. To explore the cellular mechanisms of this disease, we evaluated the pulmonary vascular responses to chronic ..
  24. Beppu H, Minowa O, Miyazono K, Kawabata M. cDNA cloning and genomic organization of the mouse BMP type II receptor. Biochem Biophys Res Commun. 1997;235:499-504 pubmed
    ..Knowledge of the structure of the BMPR-II gene is essential for the understanding of the role of bone morphogenetic proteins in the developmental and physiological processes of animals. ..
  25. Hagen M, Fagan K, Steudel W, Carr M, Lane K, Rodman D, et al. Interaction of interleukin-6 and the BMP pathway in pulmonary smooth muscle. Am J Physiol Lung Cell Mol Physiol. 2007;292:L1473-9 pubmed
    ..arterial hypertension (PAH) cases are caused by mutations in the type 2 bone morphogenetic protein receptor (BMPR2)...
  26. Long L, MacLean M, Jeffery T, Morecroft I, Yang X, Rudarakanchana N, et al. Serotonin increases susceptibility to pulmonary hypertension in BMPR2-deficient mice. Circ Res. 2006;98:818-27 pubmed
    ..We investigated the pulmonary circulation of mice deficient in BMPR-II (BMPR2(+/-) mice) and show that pulmonary hemodynamics and vascular morphometry of BMPR2(+/-) mice were similar to wild-..
  27. Beppu H, Malhotra R, Beppu Y, Lepore J, Parmacek M, Bloch K. BMP type II receptor regulates positioning of outflow tract and remodeling of atrioventricular cushion during cardiogenesis. Dev Biol. 2009;331:167-75 pubmed publisher
    ..To investigate the role of the BMP type II receptor (BMPRII) in heart development, the BMPRII gene was deleted throughout the embryo during gastrulation using ..
  28. Morty R, Nejman B, Kwapiszewska G, Hecker M, Zakrzewicz A, Kouri F, et al. Dysregulated bone morphogenetic protein signaling in monocrotaline-induced pulmonary arterial hypertension. Arterioscler Thromb Vasc Biol. 2007;27:1072-8 pubmed
    Mutations in the bmpr2 gene, encoding the type II bone morphogenetic protein (BMP) receptor, have been identified in patients with pulmonary arterial hypertension (PAH), implicating BMP signaling in PAH...
  29. Johnson J, Hemnes A, Perrien D, Schuster M, Robinson L, Gladson S, et al. Cytoskeletal defects in Bmpr2-associated pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol. 2012;302:L474-84 pubmed publisher
    ..arterial hypertension (PAH) is typically caused by a mutation in bone morphogenic protein receptor type 2 (BMPR2), and mice expressing Bmpr2 mutations develop PAH with features similar to human disease...
  30. Gomez Arroyo J, Saleem S, Mizuno S, Syed A, Bogaard H, Abbate A, et al. A brief overview of mouse models of pulmonary arterial hypertension: problems and prospects. Am J Physiol Lung Cell Mol Physiol. 2012;302:L977-91 pubmed publisher
    ..Anatomical, cellular, and genetic differences distinguish mice and rats, and pharmacogenomics may explain the degree of PH and the particular mode of pulmonary vascular adaptation and also the response of the right ventricle. ..
  31. Diebold I, Hennigs J, Miyagawa K, Li C, Nickel N, Kaschwich M, et al. BMPR2 preserves mitochondrial function and DNA during reoxygenation to promote endothelial cell survival and reverse pulmonary hypertension. Cell Metab. 2015;21:596-608 pubmed publisher
    Mitochondrial dysfunction, inflammation, and mutant bone morphogenetic protein receptor 2 (BMPR2) are associated with pulmonary arterial hypertension (PAH), an incurable disease characterized by pulmonary arterial (PA) endothelial cell (..
  32. Parikh V, Jin R, Rabello S, Gulbahce N, White K, Hale A, et al. MicroRNA-21 integrates pathogenic signaling to control pulmonary hypertension: results of a network bioinformatics approach. Circulation. 2012;125:1520-32 pubmed publisher
    ..Furthermore, this study highlights the unique utility of network biology for identifying disease-modifying microRNA in PH. ..
  33. Sengle G, Ono R, Lyons K, Bachinger H, Sakai L. A new model for growth factor activation: type II receptors compete with the prodomain for BMP-7. J Mol Biol. 2008;381:1025-39 pubmed publisher
  34. Wang S, Huang H, Chen S, Li X, Zhang W, Tang Q. Gdf6 induces commitment of pluripotent mesenchymal C3H10T1/2 cells to the adipocyte lineage. FEBS J. 2013;280:2644-51 pubmed publisher
    ..The type I receptor Bmpr1a and the type II receptors Bmpr2 and Acvr2a mediate the Gdf6 signaling pathway...
  35. Lowery J, Intini G, Gamer L, Lotinun S, Salazar V, Ote S, et al. Loss of BMPR2 leads to high bone mass due to increased osteoblast activity. J Cell Sci. 2015;128:1308-15 pubmed publisher
    ..The type 2 receptors ACVR2A and ACVR2B bind BMPs and activins but the type 2 receptor BMPR2 only binds BMPs, suggesting that type 2 receptor utilization might play a role in mediating the interaction of ..
  36. Nakamura Y, Wakitani S, Nakayama J, Wakabayashi S, Horiuchi H, Takaoka K. Temporal and spatial expression profiles of BMP receptors and noggin during BMP-2-induced ectopic bone formation. J Bone Miner Res. 2003;18:1854-62 pubmed
    ..The coordinate expression of these positive and negative regulators of BMP signaling points to a potential regulatory mechanism for bone induction. ..
  37. Mushaben E, Hershey G, Pauciulo M, Nichols W, Le Cras T. Chronic allergic inflammation causes vascular remodeling and pulmonary hypertension in BMPR2 hypomorph and wild-type mice. PLoS ONE. 2012;7:e32468 pubmed publisher
    Loss-of-function mutations in the bone morphogenetic protein receptor type 2 (BMPR2) gene have been identified in patients with heritable pulmonary arterial hypertension (PAH); however, disease penetrance is low, suggesting additional ..
  38. McBrayer Z, Dimova J, Pisansky M, Sun M, Beppu H, Gewirtz J, et al. Forebrain-Specific Loss of BMPRII in Mice Reduces Anxiety and Increases Object Exploration. PLoS ONE. 2015;10:e0139860 pubmed publisher
    To investigate the role of Bone Morphogenic Protein Receptor Type II (BMPRII) in learning, memory, and exploratory behavior in mice, a tissue-specific knockout of BMPRII in the post-natal hippocampus and forebrain was generated...
  39. Lai Y, Potoka K, Champion H, Mora A, Gladwin M. Pulmonary arterial hypertension: the clinical syndrome. Circ Res. 2014;115:115-30 pubmed publisher
  40. Knosp W, Scott V, Bachinger H, Stadler H. HOXA13 regulates the expression of bone morphogenetic proteins 2 and 7 to control distal limb morphogenesis. Development. 2004;131:4581-92 pubmed
  41. Tischfield M, Robson C, Gilette N, Chim S, Sofela F, DeLisle M, et al. Cerebral Vein Malformations Result from Loss of Twist1 Expression and BMP Signaling from Skull Progenitor Cells and Dura. Dev Cell. 2017;42:445-461.e5 pubmed publisher
  42. Kelly C, Thymiakou E, Dixon J, Tanaka S, Godwin J, Episkopou V. Rnf165/Ark2C enhances BMP-Smad signaling to mediate motor axon extension. PLoS Biol. 2013;11:e1001538 pubmed publisher
    ..Together the above data reveal an involvement of BMP-Smad signaling in motor axon advancement. ..
  43. Gorecki D, Geng Y, Thomas K, Hunt S, Barnard E, Barnard P. Expression of the dystrophin gene in mouse and rat brain. Neuroreport. 1991;2:773-6 pubmed
    ..We find the gene strongly expressed in the hippocampus and cerebral cortex, areas with an established cognitive function, and also in the Purkinje cells of the cerebellum, an area associated with motor coordination. ..
  44. Hager Theodorides A, Outram S, Shah D, Sacedon R, Shrimpton R, Vicente A, et al. Bone morphogenetic protein 2/4 signaling regulates early thymocyte differentiation. J Immunol. 2002;169:5496-504 pubmed
    ..Our study suggests that the BMP2/4 pathway may function in thymic homeostasis by regulating T cell lineage commitment and differentiation. ..
  45. Roelen B, Goumans M, Van Rooijen M, Mummery C. Differential expression of BMP receptors in early mouse development. Int J Dev Biol. 1997;41:541-9 pubmed
    ..In postimplantation embryos BMPR-II transcripts were first detected from 6.0 days post coitum. In situ hybridization analysis revealed that BMPR-II mRNA is ubiquitously expressed in the entire embryo at least until midgestation. ..
  46. Tilleman H, Hakim V, Novikov O, Liser K, Nashelsky L, Di Salvio M, et al. Bmp5/7 in concert with the mid-hindbrain organizer control development of noradrenergic locus coeruleus neurons. Mol Cell Neurosci. 2010;45:1-11 pubmed publisher
    ..In addition, we conclude that the position of the mid-hindbrain organizer determines the size of the LC and propose that Bmp5/7 play an important role in mediating this organizer function. ..
  47. Zhou W, Yu L, Fan J, Wan B, Jiang T, Yin J, et al. Endogenous Parathyroid Hormone Promotes Fracture Healing by Increasing Expression of BMPR2 through cAMP/PKA/CREB Pathway in Mice. Cell Physiol Biochem. 2017;42:551-563 pubmed publisher
    ..that expression levels of Runt-related transcription factor (RUNX2), bone morphogenetic protein-receptor-type ? (BMPR2), phosphorylated Smad 1/5/8, and phosphorylated cyclic adenosine monophosphate-responsive element binding protein (..
  48. Perez V, Ali Z, Alastalo T, Ikeno F, Sawada H, Lai Y, et al. BMP promotes motility and represses growth of smooth muscle cells by activation of tandem Wnt pathways. J Cell Biol. 2011;192:171-88 pubmed publisher
    ..We speculate that, in response to injury, factors that subvert BMP-2-mediated tandem activation of Wnt-?C and Wnt-PCP pathways contribute to obliterative vascular disease in both the systemic and pulmonary circulations. ..
  49. Alejandre Alcazar M, Shalamanov P, Amarie O, Sevilla Pérez J, Seeger W, Eickelberg O, et al. Temporal and spatial regulation of bone morphogenetic protein signaling in late lung development. Dev Dyn. 2007;236:2825-35 pubmed
    ..We describe changes in the expression and localization of BMP receptors (Bmpr1a, Bmpr1b, and Bmpr2) and Smad (Smad1, Smad4, Smad5, and Smad8) intracellular signaling proteins during the saccular and ..
  50. Talati M, West J, Blackwell T, Loyd J, Meyrick B. BMPR2 mutation alters the lung macrophage endothelin-1 cascade in a mouse model and patients with heritable pulmonary artery hypertension. Am J Physiol Lung Cell Mol Physiol. 2010;299:L363-73 pubmed publisher
    ..PAH) and in particular to heritable PAH (HPAH) with known bone morphogenetic protein type 2 receptor (BMPR2) mutations is not known...
  51. Niessen K, Zhang G, Ridgway J, Chen H, Yan M. ALK1 signaling regulates early postnatal lymphatic vessel development. Blood. 2010;115:1654-61 pubmed publisher
    ..Thus, our study reveals a novel aspect of ALK1 signaling in regulating lymphatic development and suggests that targeting ALK1 pathway might provide additional control of lymphangiogenesis in human diseases. ..
  52. Kamiya Y, Miyazono K, Miyazawa K. Smad7 inhibits transforming growth factor-beta family type i receptors through two distinct modes of interaction. J Biol Chem. 2010;285:30804-13 pubmed publisher
    ..Smad7 thus has an additional mode of interaction with TGF-? family type I receptors not possessed by Smad6, which may play roles in mediating the inhibitory effects unique to Smad7. ..
  53. Graham S, Wicher K, Jedrusik A, Guo G, Herath W, Robson P, et al. BMP signalling regulates the pre-implantation development of extra-embryonic cell lineages in the mouse embryo. Nat Commun. 2014;5:5667 pubmed publisher
    ..We investigate the role of BMP signalling by expressing dominant negative forms of Smad4 and Bmpr2, by downregulating the pathway using RNA interference against BMP ligands and by applying three different BMP ..
  54. Zhang Y, Chen W, Huang G. [Expression of bone morphogenetic protein receptor 2 in myocardialization of cardiac proximal outflow tract septum in connexin 43 knockout embryonic mice]. Zhonghua Yi Xue Za Zhi. 2011;91:2211-5 pubmed
    ..Bone morphogenetic protein receptor 2 (Bmpr2) and ?-sarcomeric acti (?-SCA) were detected by immunohistochemistry...
  55. Jiang F, Harrison L. Transient Impairment of Islet Architectural Development in Pancreas-Specific Bmpr1a-Deleted Prenatal Mice Involves Reduced Expression of E-Cadherin. Stem Cells Dev. 2017;: pubmed publisher
    ..We thus conclude that BMP signaling transiently regulates the expression of E-cad and the establishment of prenatal islet architecture. ..
  56. Kashima R, Redmond P, Ghatpande P, Roy S, Kornberg T, Hanke T, et al. Hyperactive locomotion in a Drosophila model is a functional readout for the synaptic abnormalities underlying fragile X syndrome. Sci Signal. 2017;10: pubmed publisher
    ..of LIM kinase 1 (LIMK1) downstream of augmented synthesis of bone morphogenetic protein (BMP) type 2 receptor (BMPR2) promotes aberrant synaptic development in mouse and Drosophila models of FXS and that these molecular and ..
  57. Sui X, Li D, Qiu H, Gaussin V, Depre C. Activation of the bone morphogenetic protein receptor by H11kinase/Hsp22 promotes cardiac cell growth and survival. Circ Res. 2009;104:887-95 pubmed publisher
    ..Therefore, potentiation of the BMP receptor by H11K promotes an activation of the PI3K/Akt pathway mediated by TAK1, which dictates the physiological effects of H11K on cardiac cell growth and survival. ..
  58. Fujimoto M, Ohte S, Osawa K, Miyamoto A, Tsukamoto S, Mizuta T, et al. Mutant activin-like kinase 2 in fibrodysplasia ossificans progressiva are activated via T203 by BMP type II receptors. Mol Endocrinol. 2015;29:140-52 pubmed publisher
    ..These findings suggest that the mutant ALK2 related to FOP is enhanced by BMP type II receptors via the T203-regulated phosphorylation of ALK2. ..
  59. Burton V, Holmes A, Ciuclan L, Robinson A, Roger J, Jarai G, et al. Attenuation of leukocyte recruitment via CXCR1/2 inhibition stops the progression of PAH in mice with genetic ablation of endothelial BMPR-II. Blood. 2011;118:4750-8 pubmed publisher
    ..Here we demonstrate that, in mice with endothelial-specific loss of BMPR-II expression (L1Cre(+);Bmpr2(f/f)), reduction in barrier function and the resultant pulmonary hypertension observed in vivo are the result of ..
  60. Han R, Beppu H, Lee Y, Georgopoulos K, Larue L, Li E, et al. A pair of transmembrane receptors essential for the retention and pigmentation of hair. Genesis. 2012;50:783-800 pubmed publisher
    ..Hair follicles are simple, accessible models for many developmental processes. Here, using mutant mice, we show that Bmpr2, a known receptor for bone morphogenetic proteins (Bmps), and Acvr2a, a known receptor for Bmps and activins, are ..
  61. Zakrzewicz A, Hecker M, Marsh L, Kwapiszewska G, Nejman B, Long L, et al. Receptor for activated C-kinase 1, a novel interaction partner of type II bone morphogenetic protein receptor, regulates smooth muscle cell proliferation in pulmonary arterial hypertension. Circulation. 2007;115:2957-68 pubmed
    ..Heterozygous mutations in BMPR2, encoding the type II bone morphogenetic protein receptor (BMPRII), were identified in PAH, suggesting that alterations to BMPRII function are involved in disease onset and/or ..
  62. Bagarova J, Vonner A, Armstrong K, Börgermann J, Lai C, Deng D, et al. Constitutively active ALK2 receptor mutants require type II receptor cooperation. Mol Cell Biol. 2013;33:2413-24 pubmed publisher
    ..Signaling via GS domain ALK2 mutants could be restored by the expression of either BMP type II receptor. The contribution of BMP type II receptors was independent of their ligand-binding or kinase function but ..
  63. Kashimada K, Pelosi E, Chen H, Schlessinger D, Wilhelm D, Koopman P. FOXL2 and BMP2 act cooperatively to regulate follistatin gene expression during ovarian development. Endocrinology. 2011;152:272-80 pubmed publisher
    ..Further, Wnt4-knockout mice showed reduced expression of Fst limited to early ovarian development, suggesting a role for WNT4 in the initiation, but not the maintenance, of Fst expression. ..
  64. Faber S, Robinson M, Makarenkova H, Lang R. Bmp signaling is required for development of primary lens fiber cells. Development. 2002;129:3727-37 pubmed
    ..We conclude that Bmp signaling is required for primary lens fiber cell differentiation and, given the asymmetry of the differentiation inhibition, that distinct differentiation stimuli may be active in different quadrants of the eye. ..
  65. Wu M, Li J, Engleka K, Zhou B, Lu M, Plotkin J, et al. Persistent expression of Pax3 in the neural crest causes cleft palate and defective osteogenesis in mice. J Clin Invest. 2008;118:2076-87 pubmed publisher
    ..These studies provide in vivo evidence for the importance of Pax3 downregulation during differentiation of multipotent neural crest precursors and cranial development. ..
  66. Durand C, Robin C, Bollerot K, Baron M, Ottersbach K, Dzierzak E. Embryonic stromal clones reveal developmental regulators of definitive hematopoietic stem cells. Proc Natl Acad Sci U S A. 2007;104:20838-43 pubmed
    ..We suggest that Bmp4 plays a relatively late role in the regulation of HSCs as they emerge in the midgestation AGM...
  67. Os rio C, Chac n P, Kisiswa L, White M, Wyatt S, Rodr guez T bar A, et al. Growth differentiation factor 5 is a key physiological regulator of dendrite growth during development. Development. 2013;140:4751-62 pubmed publisher
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    More than 200 heterozygous mutations in the type 2 BMP receptor gene, BMPR2, have been identified in patients with Heritable Pulmonary Arterial Hypertension (HPAH)...
  69. Gao X, Cao Y, Staloch D, Gonzales M, Aronson J, Chao C, et al. Bone morphogenetic protein signaling protects against cerulein-induced pancreatic fibrosis. PLoS ONE. 2014;9:e89114 pubmed publisher
    ..Mice with a deletion of BMP type II receptor (BMPR2(+/-)) were used in this study in comparison with wild-type mice...
  70. Zhang D, Mehler M, Song Q, Kessler J. Development of bone morphogenetic protein receptors in the nervous system and possible roles in regulating trkC expression. J Neurosci. 1998;18:3314-26 pubmed
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  72. Hassel S, Yakymovych M, Hellman U, Ronnstrand L, Knaus P, Souchelnytskyi S. Interaction and functional cooperation between the serine/threonine kinase bone morphogenetic protein type II receptor with the tyrosine kinase stem cell factor receptor. J Cell Physiol. 2006;206:457-67 pubmed
    ..Our data suggest that the complex formation between c-kit and BMPR-II leads to phosphorylation of BMPR-II at Ser757, which modulates BMPR-II-dependent signaling. ..
  73. Hurst L, Dunmore B, Long L, Crosby A, Al Lamki R, Deighton J, et al. TNF? drives pulmonary arterial hypertension by suppressing the BMP type-II receptor and altering NOTCH signalling. Nat Commun. 2017;8:14079 pubmed publisher
    ..Collectively, these findings identify mechanisms by which BMP and TNF? signalling contribute to disease, and suggest a tractable approach for therapeutic intervention in PAH. ..
  74. Borges L, Iacovino M, Koyano Nakagawa N, Baik J, Garry D, Kyba M, et al. Expression levels of endoglin distinctively identify hematopoietic and endothelial progeny at different stages of yolk sac hematopoiesis. Stem Cells. 2013;31:1893-901 pubmed publisher
  75. Broege A, Pham L, Jensen E, Emery A, Huang T, Stemig M, et al. Bone morphogenetic proteins signal via SMAD and mitogen-activated protein (MAP) kinase pathways at distinct times during osteoclastogenesis. J Biol Chem. 2013;288:37230-40 pubmed publisher
    To investigate the role of bone morphogenetic protein (BMP) signaling in osteoclastogenesis in vivo, we eliminated BMPRII in osteoclasts by creating a BMPRII(fl/fl);lysM-Cre mouse strain...
  76. Majka S, Hagen M, Blackwell T, Harral J, Johnson J, GENDRON R, et al. Physiologic and molecular consequences of endothelial Bmpr2 mutation. Respir Res. 2011;12:84 pubmed publisher
    ..Most hereditary PAH is associated with BMPR2 mutations...
  77. Lin C, Zhang Q, Yu S, Lin Y, Li S, Liu H, et al. miR-3065-5p regulates mouse odontoblastic differentiation partially through bone morphogenetic protein receptor type II. Biochem Biophys Res Commun. 2018;495:493-498 pubmed publisher
    ..report assay verified that miR-3065-5p could bind to the 3'UTR of bone morphogenetic protein receptor type II (BMPR2), which dramatically increased in the beginning of odontoblastic differentiation but decreased in the terminal ..
  78. Rejon C, Hancock M, Li Y, Thompson T, Hebert T, Bernard D. Activins bind and signal via bone morphogenetic protein receptor type II (BMPR2) in immortalized gonadotrope-like cells. Cell Signal. 2013;25:2717-26 pubmed publisher
    ..Bone morphogenetic proteins (BMPs) bind and signal via both BMP type II (BMPR2) and activin type II (ACVR2) receptors...
  79. Albers C, Hofstetter W, Sebald H, Sebald W, Siebenrock K, Klenke F. L51P - A BMP2 variant with osteoinductive activity via inhibition of Noggin. Bone. 2012;51:401-6 pubmed publisher
    ..The described strategy may help to decrease the amounts of exogenous BMPs currently required to stimulate bone healing. ..
  80. Lebeurrier N, Launay S, Macrez R, Maubert E, Legros H, Leclerc A, et al. Anti-Mullerian-hormone-dependent regulation of the brain serine-protease inhibitor neuroserpin. J Cell Sci. 2008;121:3357-65 pubmed publisher
    ..This study demonstrates the existence of an AMH-dependent signalling pathway in the brain leading to an overexpression of the serine-protease inhibitor, neuroserpin, and neuronal survival. ..
  81. Morgan E, Nguyen S, Scott V, Stadler H. Loss of Bmp7 and Fgf8 signaling in Hoxa13-mutant mice causes hypospadia. Development. 2003;130:3095-109 pubmed
    ..Finally, a novel role for Hoxa13 in the vascularization of the glans penis is also identified. ..
  82. Fernández Lloris R, Vinals F, Lopez Rovira T, Harley V, Bartrons R, Rosa J, et al. Induction of the Sry-related factor SOX6 contributes to bone morphogenetic protein-2-induced chondroblastic differentiation of C3H10T1/2 cells. Mol Endocrinol. 2003;17:1332-43 pubmed
    ..Taken together, these results indicate that SOX6 is an important downstream mediator of BMP-2 signaling in chondrogenesis. ..
  83. Hines E, Verheyden J, Lashua A, Larson S, Branchfield K, Domyan E, et al. Syndactyly in a novel Fras1(rdf) mutant results from interruption of signals for interdigital apoptosis. Dev Dyn. 2016;245:497-507 pubmed publisher
  84. Liu H, Zhang R, Chen D, Oyajobi B, Zhao M. Functional redundancy of type II BMP receptor and type IIB activin receptor in BMP2-induced osteoblast differentiation. J Cell Physiol. 2012;227:952-63 pubmed publisher
    ..The observed functional redundancy of type II BMP receptors in osteoblasts is novel information about the BMP signaling pathway essential for initiating osteoblast differentiation. ..
  85. Yang C, Yang L, Wan M, Cao X. Generation of a mouse model with expression of bone morphogenetic protein type II receptor lacking the cytoplasmic domain in osteoblasts. Ann N Y Acad Sci. 2010;1192:286-91 pubmed publisher
    ..Here we established a mouse model in which a C-terminal truncated BMP type II receptor (BMPRII) is expressed specifically in osteoblasts as a dominant negative form in the BMP/Smad signaling ..
  86. Schreuder H, Liesum A, Pohl J, Kruse M, Koyama M. Crystal structure of recombinant human growth and differentiation factor 5: evidence for interaction of the type I and type II receptor-binding sites. Biochem Biophys Res Commun. 2005;329:1076-86 pubmed