{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T12:31:26Z","timestamp":1762950686942,"version":"3.45.0"},"reference-count":48,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T00:00:00Z","timestamp":1762905600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T00:00:00Z","timestamp":1762905600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"DOI":"10.13039\/501100000274","name":"British Heart Foundation","doi-asserted-by":"publisher","award":["RG\/19\/3\/34265","RG71070 and RG8455","FS\/18\/52\/33808","SP\/12\/12\/29836"],"award-info":[{"award-number":["RG\/19\/3\/34265","RG71070 and RG8455","FS\/18\/52\/33808","SP\/12\/12\/29836"]}],"id":[{"id":"10.13039\/501100000274","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100000265","name":"Medical Research Council","doi-asserted-by":"publisher","award":["MR\/K020919\/1"],"award-info":[{"award-number":["MR\/K020919\/1"]}],"id":[{"id":"10.13039\/501100000265","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001691","name":"Japan Society for the Promotion of Science","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001691","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Angiogenesis"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Deleterious mutations in the\n                    <jats:italic>GDF2<\/jats:italic>\n                    gene, encoding BMP9, are causative of pulmonary arterial hypertension and hereditary haemorrhagic telangiectasia. Paradoxically, BMP9 germ-line knockout (\n                    <jats:italic>Gdf2<\/jats:italic>\n                    <jats:sup>\u2212\/\u2212<\/jats:sup>\n                    ;\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO) and double\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO\n                    <jats:italic>\/<\/jats:italic>\n                    conditional\n                    <jats:italic>Bmp10<\/jats:italic>\n                    cKO (dKO) mice exhibit an attenuated response to PAH-inducing stimuli. We asked whether this contradiction is due to the pathological, physiological, or genetic consequences of BMP9 knockout. In\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO mice we observed reduced pulmonary vascular smooth muscle cell (SMC) coverage and using RNA-seq analysis of\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO mouse lungs identified two novel genes,\n                    <jats:italic>COLQ<\/jats:italic>\n                    and\n                    <jats:italic>ITGA6<\/jats:italic>\n                    , which were differentially regulated in a human PAH RNA-seq dataset. In order to study BMP10 loss, postnatal tamoxifen treatment was required to induce\n                    <jats:italic>Bmp10<\/jats:italic>\n                    cKO. As previously reported, in dKO mice we observed cardiomegaly and splenomegaly, as well as hyperplasia and hemosiderosis in the pulmonary vasculature. Surprisingly, tamoxifen treated\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO control mice phenocopied these pathological changes in dKO mice and downregulated SMC marker gene transcription. Loss of BMP10 is not critical for severe tissue remodelling in the lung, heart, and spleen, rather\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO mice appear sensitive to tamoxifen and BMP9 loss is the primary cause of mild vessel remodelling due to a basal reduction of smooth muscle cell coverage. This study suggests that interaction of the BMP pathway with tamoxifen needs to be carefully considered when studying\n                    <jats:italic>Bmp9<\/jats:italic>\n                    KO mice and urges caution in the context of tamoxifen use when studying cardiovascular and pulmonary disease models.\n                  <\/jats:p>","DOI":"10.1007\/s10456-025-10017-5","type":"journal-article","created":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T12:29:06Z","timestamp":1762950546000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["BMP9 knockout impairs pulmonary vessel muscularisation and confers aberrant tamoxifen sensitivity"],"prefix":"10.1007","volume":"29","author":[{"given":"Benjamin J.","family":"Dunmore","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stephen","family":"Moore","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rowena J.","family":"Jones","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Joshua","family":"Hodgson","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kathryn","family":"Auckland","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark","family":"Southwood","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nichola","family":"Figg","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nobuhiro","family":"Kikuchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Martin","family":"Bennett","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Allan","family":"Lawrie","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christopher J.","family":"Rhodes","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark R.","family":"Toshner","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stefan","family":"Gr\u00e4f","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wei","family":"Li","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nicholas W.","family":"Morrell","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Paul D.","family":"Upton","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"UK National Cohort Study of Idiopathic and Heritable PAH Consortium","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"name":"the Uniphy Clinical Trials Network","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2025,11,12]]},"reference":[{"issue":"16","key":"10017_CR1","doi-asserted-by":"publisher","first-page":"1655","DOI":"10.1056\/NEJMra035488","volume":"351","author":"HW Farber","year":"2004","unstructured":"Farber HW, Loscalzo J (2004) Pulmonary arterial hypertension. N Engl J Med 351(16):1655\u20131665. https:\/\/doi.org\/10.1056\/NEJMra035488","journal-title":"N Engl J Med"},{"key":"10017_CR2","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.j5492","volume":"360","author":"T Thenappan","year":"2018","unstructured":"Thenappan T, Ormiston ML, Ryan JJ, Archer SL (2018) Pulmonary arterial hypertension: pathogenesis and clinical management. BMJ 360:j5492. https:\/\/doi.org\/10.1136\/bmj.j5492","journal-title":"BMJ"},{"issue":"1","key":"10017_CR3","doi-asserted-by":"publisher","first-page":"81","DOI":"10.1038\/79226","volume":"26","author":"KB Lane","year":"2000","unstructured":"Lane KB, Machado RD, Pauciulo MW, Thomson JR, Phillips JA 3rd, Loyd JE, Nichols WC, Trembath RC (2000) Heterozygous germline mutations in BMPR2, encoding a TGF-\u03b2 receptor, cause familial primary pulmonary hypertension. Nat Genet 26(1):81\u201384. https:\/\/doi.org\/10.1038\/79226","journal-title":"Nat Genet"},{"issue":"3","key":"10017_CR4","doi-asserted-by":"publisher","first-page":"737","DOI":"10.1086\/303059","volume":"67","author":"Z Deng","year":"2000","unstructured":"Deng Z, Morse JH, Slager SL, Cuervo N, Moore KJ, Venetos G, Kalachikov S, Cayanis E, Fischer SG, Barst RJ, Hodge SE, Knowles JA (2000) Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 67(3):737\u2013744. https:\/\/doi.org\/10.1086\/303059","journal-title":"Am J Hum Genet"},{"issue":"1","key":"10017_CR5","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-018-03672-4","volume":"9","author":"S Graf","year":"2018","unstructured":"Graf S, Haimel M, Bleda M, Hadinnapola C, Southgate L, Li W, Hodgson J, Liu B, Salmon RM, Southwood M, Machado RD, Martin JM, Treacy CM, Yates K, Daugherty LC, Shamardina O, Whitehorn D, Holden S, Aldred M, Bogaard HJ, Church C, Coghlan G, Condliffe R, Corris PA, Danesino C, Eyries M, Gall H, Ghio S, Ghofrani HA, Gibbs JSR, Girerd B, Houweling AC, Howard L, Humbert M, Kiely DG, Kovacs G, MacKenzie Ross RV, Moledina S, Montani D, Newnham M, Olschewski A, Olschewski H, Peacock AJ, Pepke-Zaba J, Prokopenko I, Rhodes CJ, Scelsi L, Seeger W, Soubrier F, Stein DF, Suntharalingam J, Swietlik EM, Toshner MR, van Heel DA, Vonk Noordegraaf A, Waisfisz Q, Wharton J, Wort SJ, Ouwehand WH, Soranzo N, Lawrie A, Upton PD, Wilkins MR, Trembath RC, Morrell NW (2018) Identification of rare sequence variation underlying heritable pulmonary arterial hypertension. Nat Commun 9(1):1416. https:\/\/doi.org\/10.1038\/s41467-018-03672-4","journal-title":"Nat Commun"},{"key":"10017_CR6","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.01609-2018","author":"XJ Wang","year":"2019","unstructured":"Wang XJ, Lian TY, Jiang X, Liu SF, Li SQ, Jiang R, Wu WH, Ye J, Cheng CY, Du Y, Xu XQ, Wu Y, Peng FH, Sun K, Mao YM, Yu H, Liang C, Shyy JY, Zhang SY, Zhang X, Jing ZC (2019) Germline BMP9 mutation causes idiopathic pulmonary arterial hypertension. Eur Respir J. https:\/\/doi.org\/10.1183\/13993003.01609-2018","journal-title":"Eur Respir J"},{"issue":"17","key":"10017_CR7","doi-asserted-by":"publisher","first-page":"1907","DOI":"10.1182\/blood.2019004560","volume":"136","author":"CL Shovlin","year":"2020","unstructured":"Shovlin CL, Simeoni I, Downes K, Frazer ZC, Megy K, Bernabeu-Herrero ME, Shurr A, Brimley J, Patel D, Kell L, Stephens J, Turbin IG, Aldred MA, Penkett CJ, Ouwehand WH, Jovine L, Turro E (2020) Mutational and phenotypic characterization of hereditary hemorrhagic telangiectasia. Blood 136(17):1907\u20131918. https:\/\/doi.org\/10.1182\/blood.2019004560","journal-title":"Blood"},{"issue":"9412","key":"10017_CR8","doi-asserted-by":"publisher","first-page":"852","DOI":"10.1016\/S0140-6736(04)15732-2","volume":"363","author":"CJ Gallione","year":"2004","unstructured":"Gallione CJ, Repetto GM, Legius E, Rustgi AK, Schelley SL, Tejpar S, Mitchell G, Drouin E, Westermann CJ, Marchuk DA (2004) A combined syndrome of juvenile polyposis and hereditary haemorrhagic telangiectasia associated with mutations in MADH4 (SMAD4). Lancet 363(9412):852\u2013859. https:\/\/doi.org\/10.1016\/S0140-6736(04)15732-2","journal-title":"Lancet"},{"issue":"7","key":"10017_CR9","doi-asserted-by":"publisher","first-page":"607","DOI":"10.1097\/GIM.0b013e3182136d32","volume":"13","author":"J McDonald","year":"2011","unstructured":"McDonald J, Bayrak-Toydemir P, Pyeritz RE (2011) Hereditary hemorrhagic telangiectasia: an overview of diagnosis, management, and pathogenesis. Genet Med 13(7):607\u2013616. https:\/\/doi.org\/10.1097\/GIM.0b013e3182136d32","journal-title":"Genet Med"},{"issue":"4","key":"10017_CR10","doi-asserted-by":"publisher","first-page":"315","DOI":"10.1159\/000479632","volume":"94","author":"S Dupuis-Girod","year":"2017","unstructured":"Dupuis-Girod S, Cottin V, Shovlin CL (2017) The lung in hereditary hemorrhagic telangiectasia. Respiration 94(4):315\u2013330. https:\/\/doi.org\/10.1159\/000479632","journal-title":"Respiration"},{"issue":"1","key":"10017_CR11","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1097\/MD.0b013e31802f8da1","volume":"86","author":"V Cottin","year":"2007","unstructured":"Cottin V, Chinet T, Lavole A, Corre R, Marchand E, Reynaud-Gaubert M, Plauchu H, Cordier JF, Groupe d\u2019Etudes et de Recherche sur les Maladies \"Orphelines P (2007) Pulmonary arteriovenous malformations in hereditary hemorrhagic telangiectasia: a series of 126 patients. Medicine (Baltimore) 86(1):1\u201317. https:\/\/doi.org\/10.1097\/MD.0b013e31802f8da1","journal-title":"Medicine (Baltimore)"},{"issue":"5","key":"10017_CR12","doi-asserted-by":"publisher","first-page":"1953","DOI":"10.1182\/blood-2006-07-034124","volume":"109","author":"L David","year":"2007","unstructured":"David L, Mallet C, Mazerbourg S, Feige JJ, Bailly S (2007) Identification of BMP9 and BMP10 as functional activators of the orphan activin receptor-like kinase 1 (ALK1) in endothelial cells. Blood 109(5):1953\u20131961. https:\/\/doi.org\/10.1182\/blood-2006-07-034124","journal-title":"Blood"},{"issue":"7","key":"10017_CR13","doi-asserted-by":"publisher","first-page":"777","DOI":"10.1038\/nm.3877","volume":"21","author":"L Long","year":"2015","unstructured":"Long L, Ormiston ML, Yang X, Southwood M, Graf S, Machado RD, Mueller M, Kinzel B, Yung LM, Wilkinson JM, Moore SD, Drake KM, Aldred MA, Yu PB, Upton PD, Morrell NW (2015) Selective enhancement of endothelial BMPR-II with BMP9 reverses pulmonary arterial hypertension. Nat Med 21(7):777\u2013785. https:\/\/doi.org\/10.1038\/nm.3877","journal-title":"Nat Med"},{"issue":"2","key":"10017_CR14","doi-asserted-by":"publisher","first-page":"313","DOI":"10.1007\/s00018-011-0751-1","volume":"69","author":"M Bidart","year":"2012","unstructured":"Bidart M, Ricard N, Levet S, Samson M, Mallet C, David L, Subileau M, Tillet E, Feige JJ, Bailly S (2012) BMP9 is produced by hepatocytes and circulates mainly in an active mature form complexed to its prodomain. Cell Mol Life Sci 69(2):313\u2013324. https:\/\/doi.org\/10.1007\/s00018-011-0751-1","journal-title":"Cell Mol Life Sci"},{"issue":"6","key":"10017_CR15","doi-asserted-by":"publisher","first-page":"2954","DOI":"10.1074\/jbc.M115.683292","volume":"291","author":"H Jiang","year":"2016","unstructured":"Jiang H, Salmon RM, Upton PD, Wei Z, Lawera A, Davenport AP, Morrell NW, Li W (2016) The prodomain-bound form of bone morphogenetic protein 10 is biologically active on endothelial cells. J Biol Chem 291(6):2954\u20132966. https:\/\/doi.org\/10.1074\/jbc.M115.683292","journal-title":"J Biol Chem"},{"issue":"8","key":"10017_CR16","doi-asserted-by":"publisher","first-page":"914","DOI":"10.1161\/CIRCRESAHA.107.165530","volume":"102","author":"L David","year":"2008","unstructured":"David L, Mallet C, Keramidas M, Lamande N, Gasc JM, Dupuis-Girod S, Plauchu H, Feige JJ, Bailly S (2008) Bone morphogenetic protein-9 is a circulating vascular quiescence factor. Circ Res 102(8):914\u2013922. https:\/\/doi.org\/10.1161\/CIRCRESAHA.107.165530","journal-title":"Circ Res"},{"issue":"11","key":"10017_CR17","doi-asserted-by":"publisher","first-page":"1419","DOI":"10.1164\/rccm.202005-1761OC","volume":"203","author":"W Li","year":"2021","unstructured":"Li W, Long L, Yang X, Tong Z, Southwood M, King R, Caruso P, Upton PD, Yang P, Bocobo GA, Nikolic I, Higuera A, Salmon RM, Jiang H, Lodge KM, Hoenderdos K, Baron RM, Yu PB, Condliffe AM, Summers C, Nourshargh S, Chilvers ER, Morrell NW (2021) Circulating BMP9 protects the pulmonary endothelium during inflammation-induced lung injury in mice. Am J Respir Crit Care Med 203(11):1419\u20131430. https:\/\/doi.org\/10.1164\/rccm.202005-1761OC","journal-title":"Am J Respir Crit Care Med"},{"issue":"8","key":"10017_CR18","doi-asserted-by":"publisher","first-page":"3302","DOI":"10.4049\/jimmunol.1601219","volume":"197","author":"SL Appleby","year":"2016","unstructured":"Appleby SL, Mitrofan CG, Crosby A, Hoenderdos K, Lodge K, Upton PD, Yates CM, Nash GB, Chilvers ER, Morrell NW (2016) Bone morphogenetic protein 9 enhances lipopolysaccharide-induced leukocyte recruitment to the vascular endothelium. J Immunol 197(8):3302\u20133314. https:\/\/doi.org\/10.4049\/jimmunol.1601219","journal-title":"J Immunol"},{"issue":"6","key":"10017_CR19","doi-asserted-by":"publisher","first-page":"846","DOI":"10.1161\/CIRCRESAHA.118.313356","volume":"124","author":"L Tu","year":"2019","unstructured":"Tu L, Desroches-Castan A, Mallet C, Guyon L, Cumont A, Phan C, Robert F, Thuillet R, Bordenave J, Sekine A, Huertas A, Ritvos O, Savale L, Feige JJ, Humbert M, Bailly S, Guignabert C (2019) Selective BMP-9 inhibition partially protects against experimental pulmonary hypertension. Circ Res 124(6):846\u2013855. https:\/\/doi.org\/10.1161\/CIRCRESAHA.118.313356","journal-title":"Circ Res"},{"issue":"7","key":"10017_CR20","doi-asserted-by":"publisher","first-page":"1805","DOI":"10.1093\/cvr\/cvab187","volume":"118","author":"C Bouvard","year":"2022","unstructured":"Bouvard C, Tu L, Rossi M, Desroches-Castan A, Berrebeh N, Helfer E, Roelants C, Liu H, Ouarne M, Chaumontel N, Mallet C, Battail C, Bikfalvi A, Humbert M, Savale L, Daubon T, Perret P, Tillet E, Guignabert C, Bailly S (2022) Different cardiovascular and pulmonary phenotypes for single- and double-knock-out mice deficient in BMP9 and BMP10. Cardiovasc Res 118(7):1805\u20131820. https:\/\/doi.org\/10.1093\/cvr\/cvab187","journal-title":"Cardiovasc Res"},{"issue":"4","key":"10017_CR21","doi-asserted-by":"publisher","first-page":"598","DOI":"10.1182\/blood-2012-12-472142","volume":"122","author":"S Levet","year":"2013","unstructured":"Levet S, Ciais D, Merdzhanova G, Mallet C, Zimmers TA, Lee SJ, Navarro FP, Texier I, Feige JJ, Bailly S, Vittet D (2013) Bone morphogenetic protein 9 (BMP9) controls lymphatic vessel maturation and valve formation. Blood 122(4):598\u2013607. https:\/\/doi.org\/10.1182\/blood-2012-12-472142","journal-title":"Blood"},{"issue":"9","key":"10017_CR22","doi-asserted-by":"publisher","first-page":"2219","DOI":"10.1242\/dev.01094","volume":"131","author":"H Chen","year":"2004","unstructured":"Chen H, Shi S, Acosta L, Li W, Lu J, Bao S, Chen Z, Yang Z, Schneider MD, Chien KR, Conway SJ, Yoder MC, Haneline LS, Franco D, Shou W (2004) BMP10 is essential for maintaining cardiac growth during murine cardiogenesis. Development 131(9):2219\u20132231. https:\/\/doi.org\/10.1242\/dev.01094","journal-title":"Development"},{"issue":"12","key":"10017_CR23","doi-asserted-by":"publisher","DOI":"10.1002\/mgg3.1685","volume":"9","author":"J Hodgson","year":"2021","unstructured":"Hodgson J, Ruiz-Llorente L, McDonald J, Quarrell O, Ugonna K, Bentham J, Mason R, Martin J, Moore D, Bergstrom K, Bayrak-Toydemir P, Wooderchak-Donahue W, Morrell NW, Condliffe R, Bernabeu C, Upton PD (2021) Homozygous GDF2 nonsense mutations result in a loss of circulating BMP9 and BMP10 and are associated with either PAH or an \u201cHHT-like\u201d syndrome in children. Mol Genet Genomic Med 9(12):e1685. https:\/\/doi.org\/10.1002\/mgg3.1685","journal-title":"Mol Genet Genomic Med"},{"issue":"5","key":"10017_CR24","doi-asserted-by":"publisher","first-page":"575","DOI":"10.1164\/rccm.201906-1141OC","volume":"201","author":"J Hodgson","year":"2020","unstructured":"Hodgson J, Swietlik EM, Salmon RM, Hadinnapola C, Nikolic I, Wharton J, Guo J, Liley J, Haimel M, Bleda M, Southgate L, Machado RD, Martin JM, Treacy CM, Yates K, Daugherty LC, Shamardina O, Whitehorn D, Holden S, Bogaard HJ, Church C, Coghlan G, Condliffe R, Corris PA, Danesino C, Eyries M, Gall H, Ghio S, Ghofrani HA, Gibbs JSR, Girerd B, Houweling AC, Howard L, Humbert M, Kiely DG, Kovacs G, Lawrie A, MacKenzie Ross RV, Moledina S, Montani D, Olschewski A, Olschewski H, Ouwehand WH, Peacock AJ, Pepke-Zaba J, Prokopenko I, Rhodes CJ, Scelsi L, Seeger W, Soubrier F, Suntharalingam J, Toshner MR, Trembath RC, Vonk Noordegraaf A, Wort SJ, Wilkins MR, Yu PB, Li W, Graf S, Upton PD, Morrell NW (2020) Characterization of GDF2 mutations and levels of BMP9 and BMP10 in pulmonary arterial hypertension. Am J Respir Crit Care Med 201(5):575\u2013585. https:\/\/doi.org\/10.1164\/rccm.201906-1141OC","journal-title":"Am J Respir Crit Care Med"},{"issue":"3","key":"10017_CR25","doi-asserted-by":"publisher","first-page":"276","DOI":"10.1038\/s41587-020-0439-x","volume":"38","author":"PA Ewels","year":"2020","unstructured":"Ewels PA, Peltzer A, Fillinger S, Patel H, Alneberg J, Wilm A, Garcia MU, Di Tommaso P, Nahnsen S (2020) The nf-core framework for community-curated bioinformatics pipelines. Nat Biotechnol 38(3):276\u2013278. https:\/\/doi.org\/10.1038\/s41587-020-0439-x","journal-title":"Nat Biotechnol"},{"issue":"4","key":"10017_CR26","doi-asserted-by":"publisher","first-page":"316","DOI":"10.1038\/nbt.3820","volume":"35","author":"P Di Tommaso","year":"2017","unstructured":"Di Tommaso P, Chatzou M, Floden EW, Barja PP, Palumbo E, Notredame C (2017) Nextflow enables reproducible computational workflows. Nat Biotechnol 35(4):316\u2013319. https:\/\/doi.org\/10.1038\/nbt.3820","journal-title":"Nat Biotechnol"},{"issue":"4","key":"10017_CR27","doi-asserted-by":"publisher","first-page":"586","DOI":"10.1164\/rccm.202003-0510OC","volume":"202","author":"CJ Rhodes","year":"2020","unstructured":"Rhodes CJ, Otero-Nunez P, Wharton J, Swietlik EM, Kariotis S, Harbaum L, Dunning MJ, Elinoff JM, Errington N, Thompson AAR, Iremonger J, Coghlan JG, Corris PA, Howard LS, Kiely DG, Church C, Pepke-Zaba J, Toshner M, Wort SJ, Desai AA, Humbert M, Nichols WC, Southgate L, Tregouet DA, Trembath RC, Prokopenko I, Graf S, Morrell NW, Wang D, Lawrie A, Wilkins MR (2020) Whole-blood RNA profiles associated with pulmonary arterial hypertension and clinical outcome. Am J Respir Crit Care Med 202(4):586\u2013594. https:\/\/doi.org\/10.1164\/rccm.202003-0510OC","journal-title":"Am J Respir Crit Care Med"},{"issue":"1","key":"10017_CR28","doi-asserted-by":"publisher","DOI":"10.1038\/s41467-021-27326-0","volume":"12","author":"S Kariotis","year":"2021","unstructured":"Kariotis S, Jammeh E, Swietlik EM, Pickworth JA, Rhodes CJ, Otero P, Wharton J, Iremonger J, Dunning MJ, Pandya D, Mascarenhas TS, Errington N, Thompson AAR, Romanoski CE, Rischard F, Garcia JGN, Yuan JX, An TS, Desai AA, Coghlan G, Lordan J, Corris PA, Howard LS, Condliffe R, Kiely DG, Church C, Pepke-Zaba J, Toshner M, Wort S, Graf S, Morrell NW, Wilkins MR, Lawrie A, Wang D, Consortium UKNPCS (2021) Biological heterogeneity in idiopathic pulmonary arterial hypertension identified through unsupervised transcriptomic profiling of whole blood. Nat Commun 12(1):7104. https:\/\/doi.org\/10.1038\/s41467-021-27326-0","journal-title":"Nat Commun"},{"key":"10017_CR29","doi-asserted-by":"publisher","DOI":"10.1016\/j.vph.2024.107381","volume":"155","author":"SL Mirza","year":"2024","unstructured":"Mirza SL, Upton PD, Hodgson J, Graf S, Morrell NW, Dunmore BJ (2024) SEMA3G regulates BMP9 inhibition of VEGF-mediated migration and network formation in pulmonary endothelial cells. Vascul Pharmacol 155:107381. https:\/\/doi.org\/10.1016\/j.vph.2024.107381","journal-title":"Vascul Pharmacol"},{"issue":"28","key":"10017_CR30","doi-asserted-by":"publisher","first-page":"10963","DOI":"10.1074\/jbc.RA118.002968","volume":"293","author":"E Tillet","year":"2018","unstructured":"Tillet E, Ouarne M, Desroches-Castan A, Mallet C, Subileau M, Didier R, Lioutsko A, Belthier G, Feige JJ, Bailly S (2018) A heterodimer formed by bone morphogenetic protein 9 (BMP9) and BMP10 provides most BMP biological activity in plasma. J Biol Chem 293(28):10963\u201310974. https:\/\/doi.org\/10.1074\/jbc.RA118.002968","journal-title":"J Biol Chem"},{"issue":"14","key":"10017_CR31","doi-asserted-by":"publisher","first-page":"1394","DOI":"10.1161\/CIRCULATIONAHA.120.047375","volume":"143","author":"L Wang","year":"2021","unstructured":"Wang L, Rice M, Swist S, Kubin T, Wu F, Wang S, Kraut S, Weissmann N, Bottger T, Wheeler M, Schneider A, Braun T (2021) Bmp9 and Bmp10 act directly on vascular smooth muscle cells for generation and maintenance of the contractile state. Circulation 143(14):1394\u20131410. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.120.047375","journal-title":"Circulation"},{"issue":"1","key":"10017_CR32","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1002\/ajmg.a.62522","volume":"188","author":"A Farhan","year":"2022","unstructured":"Farhan A, Yuan F, Partan E, Weiss CR (2022) Clinical manifestations of patients with GDF2 mutations associated with hereditary hemorrhagic telangiectasia type 5. Am J Med Genet A 188(1):199\u2013209. https:\/\/doi.org\/10.1002\/ajmg.a.62522","journal-title":"Am J Med Genet A"},{"issue":"1","key":"10017_CR33","doi-asserted-by":"publisher","first-page":"167","DOI":"10.1007\/s10456-022-09859-0","volume":"26","author":"H Choi","year":"2023","unstructured":"Choi H, Kim BG, Kim YH, Lee SJ, Lee YJ, Oh SP (2023) BMP10 functions independently from BMP9 for the development of a proper arteriovenous network. Angiogenesis 26(1):167\u2013186. https:\/\/doi.org\/10.1007\/s10456-022-09859-0","journal-title":"Angiogenesis"},{"issue":"25","key":"10017_CR34","doi-asserted-by":"publisher","first-page":"6162","DOI":"10.1182\/blood-2012-01-407593","volume":"119","author":"N Ricard","year":"2012","unstructured":"Ricard N, Ciais D, Levet S, Subileau M, Mallet C, Zimmers TA, Lee SJ, Bidart M, Feige JJ, Bailly S (2012) BMP9 and BMP10 are critical for postnatal retinal vascular remodeling. Blood 119(25):6162\u20136171. https:\/\/doi.org\/10.1182\/blood-2012-01-407593","journal-title":"Blood"},{"issue":"1","key":"10017_CR35","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1186\/s13046-018-0885-1","volume":"37","author":"M Ouarne","year":"2018","unstructured":"Ouarne M, Bouvard C, Boneva G, Mallet C, Ribeiro J, Desroches-Castan A, Soleilhac E, Tillet E, Peyruchaud O, Bailly S (2018) BMP9, but not BMP10, acts as a quiescence factor on tumor growth, vessel normalization and metastasis in a mouse model of breast cancer. J Exp Clin Cancer Res 37(1):209. https:\/\/doi.org\/10.1186\/s13046-018-0885-1","journal-title":"J Exp Clin Cancer Res"},{"issue":"3","key":"10017_CR36","doi-asserted-by":"publisher","first-page":"275","DOI":"10.1080\/19336918.2016.1264559","volume":"11","author":"N Heitzig","year":"2017","unstructured":"Heitzig N, Brinkmann BF, Koerdt SN, Rosso G, Shahin V, Rescher U (2017) Annexin A8 promotes VEGF-A driven endothelial cell sprouting. Cell Adhes Migr 11(3):275\u2013287. https:\/\/doi.org\/10.1080\/19336918.2016.1264559","journal-title":"Cell Adhes Migr"},{"issue":"8","key":"10017_CR37","doi-asserted-by":"publisher","DOI":"10.1016\/j.jbc.2023.104962","volume":"299","author":"TM Uyen Dao","year":"2023","unstructured":"Uyen Dao TM, Barbeau S, Messeant J, Della-Gaspera B, Bouceba T, Semprez F, Legay C, Dobbertin A (2023) The collagen ColQ binds to LRP4 and regulates the activation of the muscle-specific kinase-LRP4 receptor complex by agrin at the neuromuscular junction. J Biol Chem 299(8):104962. https:\/\/doi.org\/10.1016\/j.jbc.2023.104962","journal-title":"J Biol Chem"},{"issue":"Pt 3","key":"10017_CR38","doi-asserted-by":"publisher","first-page":"747","DOI":"10.1093\/brain\/awm325","volume":"131","author":"V Mihaylova","year":"2008","unstructured":"Mihaylova V, Muller JS, Vilchez JJ, Salih MA, Kabiraj MM, D\u2019Amico A, Bertini E, Wolfle J, Schreiner F, Kurlemann G, Rasic VM, Siskova D, Colomer J, Herczegfalvi A, Fabriciova K, Weschke B, Scola R, Hoellen F, Schara U, Abicht A, Lochmuller H (2008) Clinical and molecular genetic findings in COLQ-mutant congenital myasthenic syndromes. Brain 131(Pt 3):747\u2013759. https:\/\/doi.org\/10.1093\/brain\/awm325","journal-title":"Brain"},{"issue":"1","key":"10017_CR39","doi-asserted-by":"publisher","first-page":"228","DOI":"10.4103\/0028-3886.310105","volume":"69","author":"JJ Pallithanam","year":"2021","unstructured":"Pallithanam JJ, Prabhudesai SP, Naik N, Gauns S (2021) COLQ-related congenital myasthenic syndrome in a child from Western India. Neurol India 69(1):228\u2013229. https:\/\/doi.org\/10.4103\/0028-3886.310105","journal-title":"Neurol India"},{"issue":"1","key":"10017_CR40","doi-asserted-by":"publisher","first-page":"64","DOI":"10.1016\/s0006-291x(03)00911-2","volume":"306","author":"M Fukuda","year":"2003","unstructured":"Fukuda M (2003) Molecular cloning and characterization of human, rat, and mouse synaptotagmin XV. Biochem Biophys Res Commun 306(1):64\u201371. https:\/\/doi.org\/10.1016\/s0006-291x(03)00911-2","journal-title":"Biochem Biophys Res Commun"},{"issue":"10","key":"10017_CR41","doi-asserted-by":"publisher","first-page":"7629","DOI":"10.1074\/jbc.R100052200","volume":"277","author":"TC Sudhof","year":"2002","unstructured":"Sudhof TC (2002) Synaptotagmins: why so many? J Biol Chem 277(10):7629\u20137632. https:\/\/doi.org\/10.1074\/jbc.R100052200","journal-title":"J Biol Chem"},{"issue":"1","key":"10017_CR42","doi-asserted-by":"publisher","first-page":"95","DOI":"10.1016\/j.ajhg.2013.11.017","volume":"94","author":"M Ben Khelifa","year":"2014","unstructured":"Ben Khelifa M, Coutton C, Zouari R, Karaouzene T, Rendu J, Bidart M, Yassine S, Pierre V, Delaroche J, Hennebicq S, Grunwald D, Escalier D, Pernet-Gallay K, Jouk PS, Thierry-Mieg N, Toure A, Arnoult C, Ray PF (2014) Mutations in DNAH1, which encodes an inner arm heavy chain dynein, lead to male infertility from multiple morphological abnormalities of the sperm flagella. Am J Hum Genet 94(1):95\u2013104. https:\/\/doi.org\/10.1016\/j.ajhg.2013.11.017","journal-title":"Am J Hum Genet"},{"key":"10017_CR43","doi-asserted-by":"publisher","DOI":"10.3390\/ijms24054408","author":"V Kaushik","year":"2023","unstructured":"Kaushik V, Gessa L, Kumar N, Fernandes H (2023) Towards a new biomarker for diabetic retinopathy: exploring RBP3 structure and retinoids binding for functional imaging of eyes in vivo. Int J Mol Sci. https:\/\/doi.org\/10.3390\/ijms24054408","journal-title":"Int J Mol Sci"},{"key":"10017_CR44","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aau6627","author":"H Yokomizo","year":"2019","unstructured":"Yokomizo H, Maeda Y, Park K, Clermont AC, Hernandez SL, Fickweiler W, Li Q, Wang CH, Paniagua SM, Simao F, Ishikado A, Sun B, Wu IH, Katagiri S, Pober DM, Tinsley LJ, Avery RL, Feener EP, Kern TS, Keenan HA, Aiello LP, Sun JK, King GL (2019) Retinol binding protein 3 is increased in the retina of patients with diabetes resistant to diabetic retinopathy. Sci Transl Med. https:\/\/doi.org\/10.1126\/scitranslmed.aau6627","journal-title":"Sci Transl Med"},{"issue":"5","key":"10017_CR45","doi-asserted-by":"publisher","first-page":"2348","DOI":"10.4049\/jimmunol.161.5.2348","volume":"161","author":"DA Carlow","year":"1998","unstructured":"Carlow DA, Teh SJ, Teh HS (1998) Specific antiviral activity demonstrated by TGTP, a member of a new family of interferon-induced GTPases. J Immunol 161(5):2348\u20132355","journal-title":"J Immunol"},{"issue":"10","key":"10017_CR46","doi-asserted-by":"publisher","first-page":"3622","DOI":"10.1093\/brain\/awac192","volume":"145","author":"B Monogue","year":"2022","unstructured":"Monogue B, Chen Y, Sparks H, Behbehani R, Chai A, Rajic AJ, Massey A, Kleinschmidt-Demasters BK, Vermeren M, Kunath T, Beckham JD (2022) Alpha-synuclein supports type 1 interferon signalling in neurons and brain tissue. Brain 145(10):3622\u20133636. https:\/\/doi.org\/10.1093\/brain\/awac192","journal-title":"Brain"},{"key":"10017_CR47","doi-asserted-by":"publisher","DOI":"10.3390\/cancers15133466","author":"R Khademi","year":"2023","unstructured":"Khademi R, Malekzadeh H, Bahrami S, Saki N, Khademi R, Villa-Diaz LG (2023) Regulation and Functions of alpha6-Integrin (CD49f) in Cancer Biology. Cancers (Basel). https:\/\/doi.org\/10.3390\/cancers15133466","journal-title":"Cancers (Basel)"},{"key":"10017_CR48","doi-asserted-by":"publisher","DOI":"10.1161\/CIRCULATIONAHA.124.070693","author":"SE Lemay","year":"2025","unstructured":"Lemay SE, Montesinos MS, Grobs Y, Yokokawa T, Shimauchi T, Mougin M, Romanet C, Sauvaget M, Breuils-Bonnet S, Bourgeois A, Theberge C, Pelletier A, El Kabbout R, Martineau S, Yamamoto K, Akram M, Ray AS, Lippa B, Goodwin B, Lin FY, Wang H, Dowling JE, Lu M, Qiao Q, McTeague TA, Moy TI, Potus F, Provencher S, Boucherat O, Bonnet S (2025) Exploring integrin alpha5beta1 as a potential therapeutic target for pulmonary arterial hypertension: insights from comprehensive multicenter preclinical studies. Circulation. https:\/\/doi.org\/10.1161\/CIRCULATIONAHA.124.070693","journal-title":"Circulation"}],"container-title":["Angiogenesis"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10456-025-10017-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10456-025-10017-5\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10456-025-10017-5.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,11,12]],"date-time":"2025-11-12T12:29:07Z","timestamp":1762950547000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s10456-025-10017-5"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,11,12]]},"references-count":48,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2026,3]]}},"alternative-id":["10017"],"URL":"https:\/\/doi.org\/10.1007\/s10456-025-10017-5","relation":{},"ISSN":["1573-7209"],"issn-type":[{"value":"1573-7209","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,11,12]]},"assertion":[{"value":"1 April 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"15 October 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 November 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"P.D.U. is a scientific advisor to Interact Bio Ltd. N.W.M. is a founder and CEO of Interact Bio Ltd. M.R.T. is a scientific advisor to Apollo Therapeutics, Jannsen Pharmaceuticals, and Merck, Sharp and Dohme. All other authors declare no conflicts of interests. The funders had no role in the study\u2019s design, in the collection, analyses, interpretation of data or writing of the manuscript.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"5"}}