Physiologic and molecular consequences of endothelial Bmpr2 mutation

Respiratory Research

Volumn 12, Issue , 2011, Pages

  Majka, Susan ^a   Hagen, Moira ^a   Blackwell, Thomas ^c   Harral, Julie ^a   Johnson, Jennifer A ^c   Gendron, Robert ^b   Paradis, Helene ^b   Crona, Daniel ^a   Loyd, James E ^c   Nozik Grayck, Eva ^a   Stenmark, Kurt R ^a   West, James ^c  

^a UNIVERSITY OF COLORADO   (United States)

^b MEMORIAL UNIVERSITY OF NEWFOUNDLAND   (Canada)

^c VANDERBILT UNIVERSITY   (United States)

Author keywords

mouse model; Pulmonary Arterial Hypertension; pulmonary vascular disease

Indexed keywords

BONE MORPHOGENETIC PROTEIN RECEPTOR 2; CYTOKINE; ANGIOPOIETIN RECEPTOR; BMPR2 PROTEIN, MOUSE; BONE MORPHOGENETIC PROTEIN;

ANIMAL CELL; ANIMAL EXPERIMENT; ANIMAL TISSUE; APOPTOSIS; ARTICLE; CELL ADHESION; CELL CULTURE; CELL DIFFERENTIATION; CELL ISOLATION; CELL METABOLISM; CELL PROLIFERATION; CONTROLLED STUDY; ENDOTHELIUM CELL; GENE EXPRESSION; GENE MUTATION; GENE SILENCING; HEART RIGHT VENTRICLE PRESSURE; HETEROZYGOTE; HISTOPATHOLOGY; IN VIVO STUDY; INFLAMMATORY CELL; LUNG BLOOD VESSEL; MOUSE; NONHUMAN; PHENOTYPE; PULMONARY HYPERTENSION; SMOOTH MUSCLE FIBER; SYSTOLIC BLOOD PRESSURE; THROMBOCYTE ADHESION; THROMBOSIS; TRANSGENIC MOUSE; UPREGULATION; VASCULAR ENDOTHELIUM; VASCULAR SMOOTH MUSCLE; ANIMAL; GENE EXPRESSION REGULATION; GENETICS; GENOTYPE; HEART RIGHT VENTRICLE FUNCTION; HEART VENTRICLE PRESSURE; IMMUNOLOGY; INFLAMMATION; LUNG; METABOLISM; MICROVASCULATURE; MUTATION; PATHOLOGY; PATHOPHYSIOLOGY; PROMOTER REGION; SIGNAL TRANSDUCTION; VASCULARIZATION;

ANIMALS; APOPTOSIS; BONE MORPHOGENETIC PROTEIN RECEPTORS, TYPE II; BONE MORPHOGENETIC PROTEINS; CELL PROLIFERATION; CELLS, CULTURED; ENDOTHELIAL CELLS; GENE EXPRESSION REGULATION; GENOTYPE; HYPERTENSION, PULMONARY; INFLAMMATION; LUNG; MICE; MICE, TRANSGENIC; MICROVESSELS; MUTATION; PHENOTYPE; PROMOTER REGIONS, GENETIC; RECEPTOR, TIE-2; SIGNAL TRANSDUCTION; THROMBOSIS; VENTRICULAR FUNCTION, RIGHT; VENTRICULAR PRESSURE;

EID: 79959329487     PISSN: 14659921     EISSN: 1465993X     Source Type: Journal    
DOI: 10.1186/1465-9921-12-84     Document Type: Article

References (43)

1. Goodman DJ, Harrison DC, Popp RL. Echocardiographic features of primary pulmonary hypertension. Am J Cardiol 1974, 33:438-443. 10.1016/0002-9149(74)90329-4, 4273044.
2. Stewart DJ. Endothelial dysfunction in pulmonary vascular disorders. Arzneimittelforschung 1994, 44:451-454.
3. Sakao S, Tatsumi K. Vascular remodeling in pulmonary arterial hypertension: Multiple cancer-like pathways and possible treatment modalities. Int J Cardiol 2011, 147:4-12. 10.1016/j.ijcard.2010.07.003, 20692712.
4. Yang X, Long L, Reynolds P, Morrell N. Expression of mutant BMPR-II in pulmonary endothelial cells promotes apoptosis and a release of factors that stimulate proliferation of pulmonary arterial smooth muscle cells. Pulmonary Circulation 2011, 1:103-110.
5. Lane KB, Machado RD, Pauciulo MW, Thomson JR, Phillips JA, Loyd JE, Nichols WC, Trembath RC. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet 2000, 26:81-84.
6. Machado RD, Pauciulo MW, Thomson JR, Lane KB, Morgan NV, Wheeler L, Phillips J, Newman J, Williams D, Galie N, Manes A, McNeil K, Yacoub M, Mikhail G, Rogers P, Corris P, Humbert M, Donnai D, Martensson G, Tranebjaerg L, Loyd JE, Trembath RC, Nichols WC. BMPR2 haploinsufficiency as the inherited molecular mechanism for primary pulmonary hypertension. Am J Hum Genet 2001, 68:92-102. 10.1086/316947, 1234937, 11115378.
7. Rajkumar R, Konishi K, Richards TJ, Ishizawar DC, Wiechert AC, Kaminski N, Ahmad F. Genomewide RNA expression profiling in lung identifies distinct signatures in idiopathic pulmonary arterial hypertension and secondary pulmonary hypertension. Am J Physiol Heart Circ Physiol 2010, 298:H1235-1248. 10.1152/ajpheart.00254.2009, 2853417, 20081107.
8. West J. Cross talk between Smad, MAPK, and actin in the etiology of pulmonary arterial hypertension. Adv Exp Med Biol 2010, 661:265-278. 10.1007/978-1-60761-500-2_17, 20204736.
9. West J, Harral J, Lane K, Deng Y, Ickes B, Crona D, Albu S, Stewart D, Fagan K. Mice expressing BMPR2R899X transgene in smooth muscle develop pulmonary vascular lesions. Am J Physiol Lung Cell Mol Physiol 2008, 295:L744-755. 10.1152/ajplung.90255.2008, 2584890, 18723761.
10. Foletta VC, Lim MA, Soosairajah J, Kelly AP, Stanley EG, Shannon M, He W, Das S, Massague J, Bernard O. Direct signaling by the BMP type II receptor via the cytoskeletal regulator LIMK1. J Cell Biol 2003, 162:1089-1098. 10.1083/jcb.200212060, 2172847, 12963706.
11. Wong WK, Knowles JA, Morse JH. Bone morphogenetic protein receptor type II C-terminus interacts with c-Src: implication for a role in pulmonary arterial hypertension. Am J Respir Cell Mol Biol 2005, 33:438-446. 10.1165/rcmb.2005-0103OC, 2715351, 16002577.
12. Rudarakanchana N, Flanagan JA, Chen H, Upton PD, Machado R, Patel D, Trembath RC, Morrell NW. Functional analysis of bone morphogenetic protein type II receptor mutations underlying primary pulmonary hypertension. Hum Mol Genet 2002, 11:1517-1525. 10.1093/hmg/11.13.1517, 12045205.
13. Tada Y, Majka S, Carr M, Harral J, Crona D, Kuriyama T, West J. 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:L1556-1563. 10.1152/ajplung.00305.2006, 17369292.
14. West J, Fagan K, Steudel W, Fouty B, Lane K, Harral J, Hoedt-Miller M, Tada Y, Ozimek J, Tuder R, Rodman DM. Pulmonary hypertension in transgenic mice expressing a dominant-negative BMPRII gene in smooth muscle. Circ Res 2004, 94:1109-1114. 10.1161/01.RES.0000126047.82846.20, 15031260.
15. Yelle D, Deng YP, West J, Stewart DJ. Endothelial-Targeted BMPR2 Loss-of-Function Mutations Cause Increased Apoptosis and Pulmonary Arterial Hypertension. Circulation 2009, 120:S1021-S1021.
16. Wall DS, Gendron RL, Good WV, Miskiewicz E, Woodland M, Leblanc K, Paradis H. Conditional knockdown of tubedown-1 in endothelial cells leads to neovascular retinopathy. Invest Ophthalmol Vis Sci 2004, 45:3704-3712. 10.1167/iovs.03-1410, 15452080.
17. Paradis H, Islam T, Tucker S, Tao L, Koubi S, Gendron RL. Tubedown associates with cortactin and controls permeability of retinal endothelial cells to albumin. J Cell Sci 2008, 121:1965-1972. 10.1242/jcs.028597, 18495841.
18. Gendron RL, Laver NV, Good WV, Grossniklaus HE, Miskiewicz E, Whelan MA, Walker J, Paradis H. Loss of Tubedown Expression as a contributing factor in the Development of Age-Related Retinopathy. Invest Ophthalmol Vis Sci 2010,
19. Carstairs KC. The identification of platelets and platelet antigens in histological sections. J Pathol Bacteriol 1965, 90:225-231. 10.1002/path.1700900124, 5320852.
20. Jat PS, Noble MD, Ataliotis P, Tanaka Y, Yannoutsos N, Larsen L, Kioussis D. Direct derivation of conditionally immortal cell lines from an H-2Kb-tsA58 transgenic mouse. Proc Natl Acad Sci USA 1991, 88:5096-5100. 10.1073/pnas.88.12.5096, 51818, 1711218.
21. Pozzi A, Moberg PE, Miles LA, Wagner S, Soloway P, Gardner HA. Elevated matrix metalloprotease and angiostatin levels in integrin alpha 1 knockout mice cause reduced tumor vascularization. Proc Natl Acad Sci USA 2000, 97:2202-2207. 10.1073/pnas.040378497, 15778, 10681423.
22. Zhang B, Kirov S, Snoddy J. WebGestalt: an integrated system for exploring gene sets in various biological contexts. Nucleic Acids Res 2005, 33:W741-748. 10.1093/nar/gki475, 1160236, 15980575.
23. Herve P, Humbert M, Sitbon O, Parent F, Nunes H, Legal C, Garcia G, Simonneau G. Pathobiology of pulmonary hypertension. The role of platelets and thrombosis. Clin Chest Med 2001, 22:451-458. 10.1016/S0272-5231(05)70283-5, 11590840.
24. Wagenvoort CA. Pathology of pulmonary thromboembolism. Chest 1995, 107:10S-17S. 10.1378/chest.107.1_Supplement.10S, 7813322.
25. Wagenvoort CA, Mulder PG. Thrombotic lesions in primary plexogenic arteriopathy. Similar pathogenesis or complication?. Chest 1993, 103:844-849. 10.1378/chest.103.3.844, 8449079.
26. Teichert-Kuliszewska K, Kutryk MJ, Kuliszewski MA, Karoubi G, Courtman DW, Zucco L, Granton J, Stewart DJ. Bone morphogenetic protein receptor-2 signaling promotes pulmonary arterial endothelial cell survival: implications for loss-of-function mutations in the pathogenesis of pulmonary hypertension. Circ Res 2006, 98:209-217. 10.1161/01.RES.0000200180.01710.e6, 16357305.
27. Lane K, Talati M, Austin E, Hemnes A, Johnson J, Fessel J, Blackwell T, Mernaugh R, Robinson L, Fike C, Roberts Ii L, West J. Oxidative injury is a common consequence of BMPR2 mutations. Pulmonary Circulation 2011, 1:72-83.
28. Park K, Kim K, Rho SB, Choi K, Kim D, Oh SH, Park J, Lee SH, Lee JH. Homeobox Msx1 interacts with p53 tumor suppressor and inhibits tumor growth by inducing apoptosis. Cancer Res 2005, 65:749-757.
29. Kiyono M, Shibuya M. Bone morphogenetic protein 4 mediates apoptosis of capillary endothelial cells during rat pupillary membrane regression. Mol Cell Biol 2003, 23:4627-4636. 10.1128/MCB.23.13.4627-4636.2003, 164857, 12808102.
30. Bendall AJ, Rincon-Limas DE, Botas J, Abate-Shen C. Protein complex formation between Msx1 and Lhx2 homeoproteins is incompatible with DNA binding activity. Differentiation 1998, 63:151-157. 10.1046/j.1432-0436.1998.6330151.x, 9697309.
31. Jurasz P, Yurkova N, Kirshenbaum L, Stewart DJ. VEGF masks BNIP3-mediated apoptosis of hypoxic endothelial cells. Angiogenesis 2011,
32. Doumont G, Martoriati A, Marine JC. PTPRV is a key mediator of p53-induced cell cycle exit. Cell Cycle 2005, 4:1703-1705. 10.4161/cc.4.12.2207, 16258284.
33. Toumaniantz G, Ferland-McCollough D, Cario-Toumaniantz C, Pacaud P, Loirand G. The Rho protein exchange factor Vav3 regulates vascular smooth muscle cell proliferation and migration. Cardiovasc Res 2010, 86:131-140.
34. Zitzler J, Link D, Schafer R, Liebetrau W, Kazinski M, Bonin-Debs A, Behl C, Buckel P, Brinkmann U. High-throughput functional genomics identifies genes that ameliorate toxicity due to oxidative stress in neuronal HT-22 cells: GFPT2 protects cells against peroxide. Mol Cell Proteomics 2004, 3:834-840. 10.1074/mcp.M400054-MCP200, 15181156.
35. Yeh CS, Wang JY, Chung FY, Lee SC, Huang MY, Kuo CW, Yang MJ, Lin SR. Significance of the glycolytic pathway and glycolysis related-genes in tumorigenesis of human colorectal cancers. Oncol Rep 2008, 19:81-91.
36. Cohen R, Holland JP, Yokoi T, Holland MJ. Identification of a regulatory region that mediates glucose-dependent induction of the Saccharomyces cerevisiae enolase gene ENO2. Mol Cell Biol 1986, 6:2287-2297. 367781, 3537717.
37. Timmers S, Schrauwen P, de Vogel J. Muscular diacylglycerol metabolism and insulin resistance. Physiol Behav 2008, 94:242-251. 10.1016/j.physbeh.2007.12.002, 18207474.
38. Liekens S, Schols D, Hatse S. CXCL12-CXCR4 axis in angiogenesis, metastasis and stem cell mobilization. Curr Pharm Des 2010, 16:3903-3920. 10.2174/138161210794455003, 21158728.
39. Jackson SW, Hoshi T, Wu Y, Sun X, Enjyoji K, Cszimadia E, Sundberg C, Robson SC. Disordered purinergic signaling inhibits pathological angiogenesis in cd39/Entpd1-null mice. Am J Pathol 2007, 171:1395-1404. 10.2353/ajpath.2007.070190, 1988887, 17823293.
40. Burton VJ, Ciuclan LI, Holmes AM, Rodman DM, Walker C, Budd DC. Bone morphogenetic protein receptor-II regulates pulmonary artery endothelial cell barrier function. Blood 2010,
41. Hong KH, Lee YJ, Lee E, Park SO, Han C, Beppu H, Li E, Raizada MK, Bloch KD, Oh SP. Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension. Circulation 2008, 118:722-730. 10.1161/CIRCULATIONAHA.107.736801, 18663089.
42. Wagenvoort CA. Vasoconstriction and medial hypertrophy in pulmonary hypertension. Circulation 1960, 22:535-546.
43. Stenmark KR, Meyrick B, Galie N, Mooi WJ, McMurtry IF. Animal models of pulmonary arterial hypertension: the hope for etiological discovery and pharmacological cure. Am J Physiol Lung Cell Mol Physiol 2009, 297:L1013-1032. 10.1152/ajplung.00217.2009, 19748998.