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SMAD4 Deficiency Leads to Development of Arteriovenous Malformations in Neonatal and Adult Mice.

Kim YH, Choe SW, Chae MY, Hong S, Oh SP.

J Am Heart Assoc. 2018 Nov 6;7(21):e009514. doi: 10.1161/JAHA.118.009514.


Pazopanib may reduce bleeding in hereditary hemorrhagic telangiectasia.

Faughnan ME, Gossage JR, Chakinala MM, Oh SP, Kasthuri R, Hughes CCW, McWilliams JP, Parambil JG, Vozoris N, Donaldson J, Paul G, Berry P, Sprecher DL.

Angiogenesis. 2019 Feb;22(1):145-155. doi: 10.1007/s10456-018-9646-1. Epub 2018 Sep 6.


CXCL12-CXCR4 signalling plays an essential role in proper patterning of aortic arch and pulmonary arteries.

Kim BG, Kim YH, Stanley EL, Garrido-Martin EM, Lee YJ, Oh SP.

Cardiovasc Res. 2017 Nov 1;113(13):1677-1687. doi: 10.1093/cvr/cvx188.


Reduced activin receptor-like kinase 1 activity promotes cardiac fibrosis in heart failure.

Morine KJ, Qiao X, Paruchuri V, Aronovitz MJ, Mackey EE, Buiten L, Levine J, Ughreja K, Nepali P, Blanton RM, Oh SP, Karas RH, Kapur NK.

Cardiovasc Pathol. 2017 Nov - Dec;31:26-33. doi: 10.1016/j.carpath.2017.07.004. Epub 2017 Jul 18.


Conditional knockout of activin like kinase-1 (ALK-1) leads to heart failure without maladaptive remodeling.

Morine KJ, Qiao X, Paruchuri V, Aronovitz MJ, Mackey EE, Buiten L, Levine J, Ughreja K, Nepali P, Blanton RM, Karas RH, Oh SP, Kapur NK.

Heart Vessels. 2017 May;32(5):628-636. doi: 10.1007/s00380-017-0955-x. Epub 2017 Feb 17.


Mitochondrial ATP transporter depletion protects mice against liver steatosis and insulin resistance.

Cho J, Zhang Y, Park SY, Joseph AM, Han C, Park HJ, Kalavalapalli S, Chun SK, Morgan D, Kim JS, Someya S, Mathews CE, Lee YJ, Wohlgemuth SE, Sunny NE, Lee HY, Choi CS, Shiratsuchi T, Oh SP, Terada N.

Nat Commun. 2017 Feb 16;8:14477. doi: 10.1038/ncomms14477.


Smooth muscle cell-specific Tgfbr1 deficiency promotes aortic aneurysm formation by stimulating multiple signaling events.

Yang P, Schmit BM, Fu C, DeSart K, Oh SP, Berceli SA, Jiang Z.

Sci Rep. 2016 Oct 14;6:35444. doi: 10.1038/srep35444.


Effect of Topical Intranasal Therapy on Epistaxis Frequency in Patients With Hereditary Hemorrhagic Telangiectasia: A Randomized Clinical Trial.

Whitehead KJ, Sautter NB, McWilliams JP, Chakinala MM, Merlo CA, Johnson MH, James M, Everett EM, Clancy MS, Faughnan ME, Oh SP, Olitsky SE, Pyeritz RE, Gossage JR.

JAMA. 2016 Sep 6;316(9):943-51. doi: 10.1001/jama.2016.11724.


BMP9/ALK1 inhibits neovascularization in mouse models of age-related macular degeneration.

Ntumba K, Akla N, Oh SP, Eichmann A, Larrivée B.

Oncotarget. 2016 Aug 30;7(35):55957-55969. doi: 10.18632/oncotarget.11182.


Persistent infiltration and pro-inflammatory differentiation of monocytes cause unresolved inflammation in brain arteriovenous malformation.

Zhang R, Han Z, Degos V, Shen F, Choi EJ, Sun Z, Kang S, Wong M, Zhu W, Zhan L, Arthur HM, Oh SP, Faughnan ME, Su H.

Angiogenesis. 2016 Oct;19(4):451-461. doi: 10.1007/s10456-016-9519-4. Epub 2016 Jun 20.


PIERCE1 is critical for specification of left-right asymmetry in mice.

Sung YH, Baek IJ, Kim YH, Gho YS, Oh SP, Lee YJ, Lee HW.

Sci Rep. 2016 Jun 16;6:27932. doi: 10.1038/srep27932.


Interaction Between ALK1 Signaling and Connexin40 in the Development of Arteriovenous Malformations.

Gkatzis K, Thalgott J, Dos-Santos-Luis D, Martin S, Lamandé N, Carette MF, Disch F, Snijder RJ, Westermann CJ, Mager JJ, Oh SP, Miquerol L, Arthur HM, Mummery CL, Lebrin F.

Arterioscler Thromb Vasc Biol. 2016 Apr;36(4):707-17. doi: 10.1161/ATVBAHA.115.306719. Epub 2016 Jan 28.


Increasing brain angiotensin converting enzyme 2 activity decreases anxiety-like behavior in male mice by activating central Mas receptors.

Wang L, de Kloet AD, Pati D, Hiller H, Smith JA, Pioquinto DJ, Ludin JA, Oh SP, Katovich MJ, Frazier CJ, Raizada MK, Krause EG.

Neuropharmacology. 2016 Jun;105:114-123. doi: 10.1016/j.neuropharm.2015.12.026. Epub 2016 Jan 6.


Neuropilin 1 balances β8 integrin-activated TGFβ signaling to control sprouting angiogenesis in the brain.

Hirota S, Clements TP, Tang LK, Morales JE, Lee HS, Oh SP, Rivera GM, Wagner DS, McCarty JH.

Development. 2015 Dec 15;142(24):4363-73. doi: 10.1242/dev.113746. Epub 2015 Nov 19.


Angiotensin-converting enzyme 2 inhibits high-mobility group box 1 and attenuates cardiac dysfunction post-myocardial ischemia.

Qi YF, Zhang J, Wang L, Shenoy V, Krause E, Oh SP, Pepine CJ, Katovich MJ, Raizada MK.

J Mol Med (Berl). 2016 Jan;94(1):37-49.


Mouse models of hereditary hemorrhagic telangiectasia: recent advances and future challenges.

Tual-Chalot S, Oh SP, Arthur HM.

Front Genet. 2015 Feb 18;6:25. doi: 10.3389/fgene.2015.00025. eCollection 2015.


Mitochondrial ATP transporter Ant2 depletion impairs erythropoiesis and B lymphopoiesis.

Cho J, Seo J, Lim CH, Yang L, Shiratsuchi T, Lee MH, Chowdhury RR, Kasahara H, Kim JS, Oh SP, Lee YJ, Terada N.

Cell Death Differ. 2015 Sep;22(9):1437-50. doi: 10.1038/cdd.2014.230. Epub 2015 Jan 23.


Essential role for TMEM100 in vascular integrity but limited contributions to the pathogenesis of hereditary haemorrhagic telangiectasia.

Moon EH, Kim YS, Seo J, Lee S, Lee YJ, Oh SP.

Cardiovasc Res. 2015 Mar 1;105(3):353-60. doi: 10.1093/cvr/cvu260. Epub 2014 Dec 23.


Common and distinctive pathogenetic features of arteriovenous malformations in hereditary hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 animal models--brief report.

Garrido-Martin EM, Nguyen HL, Cunningham TA, Choe SW, Jiang Z, Arthur HM, Lee YJ, Oh SP.

Arterioscler Thromb Vasc Biol. 2014 Oct;34(10):2232-6. doi: 10.1161/ATVBAHA.114.303984. Epub 2014 Jul 31.


Adenine nucleotide translocase 4 is expressed within embryonic ovaries and dispensable during oogenesis.

Lim CH, Brower JV, Resnick JL, Oh SP, Terada N.

Reprod Sci. 2015 Feb;22(2):250-7. doi: 10.1177/1933719114542026. Epub 2014 Jul 16.


VEGF neutralization can prevent and normalize arteriovenous malformations in an animal model for hereditary hemorrhagic telangiectasia 2.

Han C, Choe SW, Kim YH, Acharya AP, Keselowsky BG, Sorg BS, Lee YJ, Oh SP.

Angiogenesis. 2014 Oct;17(4):823-830. doi: 10.1007/s10456-014-9436-3. Epub 2014 Jun 24.


Endothelial depletion of Acvrl1 in mice leads to arteriovenous malformations associated with reduced endoglin expression.

Tual-Chalot S, Mahmoud M, Allinson KR, Redgrave RE, Zhai Z, Oh SP, Fruttiger M, Arthur HM.

PLoS One. 2014 Jun 4;9(6):e98646. doi: 10.1371/journal.pone.0098646. eCollection 2014.


Bone morphogenetic protein-9 inhibits lymphatic vessel formation via activin receptor-like kinase 1 during development and cancer progression.

Yoshimatsu Y, Lee YG, Akatsu Y, Taguchi L, Suzuki HI, Cunha SI, Maruyama K, Suzuki Y, Yamazaki T, Katsura A, Oh SP, Zimmers TA, Lee SJ, Pietras K, Koh GY, Miyazono K, Watabe T.

Proc Natl Acad Sci U S A. 2013 Nov 19;110(47):18940-5. doi: 10.1073/pnas.1310479110. Epub 2013 Oct 16.


SnoN facilitates ALK1-Smad1/5 signaling during embryonic angiogenesis.

Zhu Q, Kim YH, Wang D, Oh SP, Luo K.

J Cell Biol. 2013 Sep 16;202(6):937-50. doi: 10.1083/jcb.201208113. Epub 2013 Sep 9.


Enhanced responses to angiogenic cues underlie the pathogenesis of hereditary hemorrhagic telangiectasia 2.

Choi EJ, Kim YH, Choe SW, Tak YG, Garrido-Martin EM, Chang M, Lee YJ, Oh SP.

PLoS One. 2013 May 10;8(5):e63138. doi: 10.1371/journal.pone.0063138. Print 2013.


Conditional deletion of Jak2 reveals an essential role in hematopoiesis throughout mouse ontogeny: implications for Jak2 inhibition in humans.

Park SO, Wamsley HL, Bae K, Hu Z, Li X, Choe SW, Slayton WB, Oh SP, Wagner KU, Sayeski PP.

PLoS One. 2013;8(3):e59675. doi: 10.1371/journal.pone.0059675. Epub 2013 Mar 27.


SMAD1 deficiency in either endothelial or smooth muscle cells can predispose mice to pulmonary hypertension.

Han C, Hong KH, Kim YH, Kim MJ, Song C, Kim MJ, Kim SJ, Raizada MK, Oh SP.

Hypertension. 2013 May;61(5):1044-52. doi: 10.1161/HYPERTENSIONAHA.111.199158. Epub 2013 Mar 11.


Reduced mural cell coverage and impaired vessel integrity after angiogenic stimulation in the Alk1-deficient brain.

Chen W, Guo Y, Walker EJ, Shen F, Jun K, Oh SP, Degos V, Lawton MT, Tihan T, Davalos D, Akassoglou K, Nelson J, Pile-Spellman J, Su H, Young WL.

Arterioscler Thromb Vasc Biol. 2013 Feb;33(2):305-10. doi: 10.1161/ATVBAHA.112.300485. Epub 2012 Dec 13.


Minimal homozygous endothelial deletion of Eng with VEGF stimulation is sufficient to cause cerebrovascular dysplasia in the adult mouse.

Choi EJ, Walker EJ, Shen F, Oh SP, Arthur HM, Young WL, Su H.

Cerebrovasc Dis. 2012;33(6):540-7. doi: 10.1159/000337762. Epub 2012 May 9.


Gastric angiodysplasia in a hereditary hemorrhagic telangiectasia type 2 patient.

Ha M, Kim YJ, Kwon KA, Hahm KB, Kim MJ, Kim DK, Lee YJ, Oh SP.

World J Gastroenterol. 2012 Apr 21;18(15):1840-4. doi: 10.3748/wjg.v18.i15.1840.


Clinical and genetic analyses of three Korean families with hereditary hemorrhagic telangiectasia.

Kim MJ, Kim ST, Lee HD, Lee KY, Seo J, Lee JB, Lee YJ, Oh SP.

BMC Med Genet. 2011 Oct 3;12:130. doi: 10.1186/1471-2350-12-130.


Restraint of angiogenesis by zinc finger transcription factor CTCF-dependent chromatin insulation.

Tang M, Chen B, Lin T, Li Z, Pardo C, Pampo C, Chen J, Lien CL, Wu L, Ai L, Wang H, Yao K, Oh SP, Seto E, Smith LE, Siemann DW, Kladde MP, Cepko CL, Lu J.

Proc Natl Acad Sci U S A. 2011 Sep 13;108(37):15231-6. doi: 10.1073/pnas.1104662108. Epub 2011 Sep 6.


TGF-β signaling in endothelial cells, but not neuroepithelial cells, is essential for cerebral vascular development.

Nguyen HL, Lee YJ, Shin J, Lee E, Park SO, McCarty JH, Oh SP.

Lab Invest. 2011 Nov;91(11):1554-63. doi: 10.1038/labinvest.2011.124. Epub 2011 Aug 29.


Daxx mediates activation-induced cell death in microglia by triggering MST1 signalling.

Yun HJ, Yoon JH, Lee JK, Noh KT, Yoon KW, Oh SP, Oh HJ, Chae JS, Hwang SG, Kim EH, Maul GG, Lim DS, Choi EJ.

EMBO J. 2011 May 13;30(12):2465-76. doi: 10.1038/emboj.2011.152.


Arteriovenous malformation in the adult mouse brain resembling the human disease.

Walker EJ, Su H, Shen F, Choi EJ, Oh SP, Chen G, Lawton MT, Kim H, Chen Y, Chen W, Young WL.

Ann Neurol. 2011 Jun;69(6):954-62. doi: 10.1002/ana.22348. Epub 2011 Mar 17.


Vascular smooth muscle Jak2 deletion prevents angiotensin II-mediated neointima formation following injury in mice.

Kirabo A, Oh SP, Kasahara H, Wagner KU, Sayeski PP.

J Mol Cell Cardiol. 2011 Jun;50(6):1026-34. doi: 10.1016/j.yjmcc.2011.03.005. Epub 2011 Mar 17.


Vascular smooth muscle Jak2 mediates angiotensin II-induced hypertension via increased levels of reactive oxygen species.

Kirabo A, Kearns PN, Jarajapu YP, Sasser JM, Oh SP, Grant MB, Kasahara H, Cardounel AJ, Baylis C, Wagner KU, Sayeski PP.

Cardiovasc Res. 2011 Jul 1;91(1):171-9. doi: 10.1093/cvr/cvr059. Epub 2011 Feb 24.


Generation of mice with a conditional and reporter allele for Tmem100.

Moon EH, Kim MJ, Ko KS, Kim YS, Seo J, Oh SP, Lee YJ.

Genesis. 2010 Nov;48(11):673-8. doi: 10.1002/dvg.20674. Epub 2010 Oct 30.


Growth differentiation factor 11 signaling controls retinoic acid activity for axial vertebral development.

Lee YJ, McPherron A, Choe S, Sakai Y, Chandraratna RA, Lee SJ, Oh SP.

Dev Biol. 2010 Nov 1;347(1):195-203. doi: 10.1016/j.ydbio.2010.08.022. Epub 2010 Aug 27.


Tnk1/Kos1: a novel tumor suppressor.

May WS, Hoare K, Hoare S, Reinhard MK, Lee YJ, Oh SP.

Trans Am Clin Climatol Assoc. 2010;121:281-92; discussion 292-3. Review.


Pinin modulates expression of an intestinal homeobox gene, Cdx2, and plays an essential role for small intestinal morphogenesis.

Joo JH, Taxter TJ, Munguba GC, Kim YH, Dhaduvai K, Dunn NW, Degan WJ, Oh SP, Sugrue SP.

Dev Biol. 2010 Sep 15;345(2):191-203. doi: 10.1016/j.ydbio.2010.07.009. Epub 2010 Jul 14.


Spectral imaging reveals microvessel physiology and function from anastomoses to thromboses.

Wankhede M, Agarwal N, Fraga-Silva RA, deDeugd C, Raizada MK, Oh SP, Sorg BS.

J Biomed Opt. 2010 Jan-Feb;15(1):011111. doi: 10.1117/1.3316299.


Impaired terminal differentiation of hippocampal granule neurons and defective contextual memory in PC3/Tis21 knockout mice.

Farioli-Vecchioli S, Saraulli D, Costanzi M, Leonardi L, Cinà I, Micheli L, Nutini M, Longone P, Oh SP, Cestari V, Tirone F.

PLoS One. 2009 Dec 17;4(12):e8339. doi: 10.1371/journal.pone.0008339.


Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia.

Park SO, Wankhede M, Lee YJ, Choi EJ, Fliess N, Choe SW, Oh SH, Walter G, Raizada MK, Sorg BS, Oh SP.

J Clin Invest. 2009 Nov;119(11):3487-96. doi: 10.1172/JCI39482. Epub 2009 Oct 1.


Prevention of pulmonary hypertension by Angiotensin-converting enzyme 2 gene transfer.

Yamazato Y, Ferreira AJ, Hong KH, Sriramula S, Francis J, Yamazato M, Yuan L, Bradford CN, Shenoy V, Oh SP, Katovich MJ, Raizada MK.

Hypertension. 2009 Aug;54(2):365-71. doi: 10.1161/HYPERTENSIONAHA.108.125468. Epub 2009 Jun 29.


Adenine nucleotide translocase 4 deficiency leads to early meiotic arrest of murine male germ cells.

Brower JV, Lim CH, Jorgensen M, Oh SP, Terada N.

Reproduction. 2009 Sep;138(3):463-70. doi: 10.1530/REP-09-0201. Epub 2009 Jun 25.


Evidence for angiotensin-converting enzyme 2 as a therapeutic target for the prevention of pulmonary hypertension.

Ferreira AJ, Shenoy V, Yamazato Y, Sriramula S, Francis J, Yuan L, Castellano RK, Ostrov DA, Oh SP, Katovich MJ, Raizada MK.

Am J Respir Crit Care Med. 2009 Jun 1;179(11):1048-54. doi: 10.1164/rccm.200811-1678OC. Epub 2009 Feb 26.


Tnk1/Kos1 knockout mice develop spontaneous tumors.

Hoare S, Hoare K, Reinhard MK, Lee YJ, Oh SP, May WS Jr.

Cancer Res. 2008 Nov 1;68(21):8723-32. doi: 10.1158/0008-5472.CAN-08-1467.


Perinatal loss of Nkx2-5 results in rapid conduction and contraction defects.

Briggs LE, Takeda M, Cuadra AE, Wakimoto H, Marks MH, Walker AJ, Seki T, Oh SP, Lu JT, Sumners C, Raizada MK, Horikoshi N, Weinberg EO, Yasui K, Ikeda Y, Chien KR, Kasahara H.

Circ Res. 2008 Sep 12;103(6):580-90. doi: 10.1161/CIRCRESAHA.108.171835. Epub 2008 Aug 8.


Genetic ablation of the BMPR2 gene in pulmonary endothelium is sufficient to predispose to pulmonary arterial hypertension.

Hong KH, Lee YJ, Lee E, Park SO, Han C, Beppu H, Li E, Raizada MK, Bloch KD, Oh SP.

Circulation. 2008 Aug 12;118(7):722-30. doi: 10.1161/CIRCULATIONAHA.107.736801. Epub 2008 Jul 28.

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