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Citations to this article

Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia
Sung Ok Park, … , Brian S. Sorg, S. Paul Oh
Sung Ok Park, … , Brian S. Sorg, S. Paul Oh
Published October 1, 2009
Citation Information: J Clin Invest. 2009;119(11):3487-3496. https://doi.org/10.1172/JCI39482.
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Technical Advance Vascular biology

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

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Abstract

Arteriovenous malformations (AVMs) are vascular anomalies where arteries and veins are directly connected through a complex, tangled web of abnormal arteries and veins instead of a normal capillary network. AVMs in the brain, lung, and visceral organs, including the liver and gastrointestinal tract, result in considerable morbidity and mortality. AVMs are the underlying cause of three major clinical symptoms of a genetic vascular dysplasia termed hereditary hemorrhagic telangiectasia (HHT), which is characterized by recurrent nosebleeds, mucocutaneous telangiectases, and visceral AVMs and caused by mutations in one of several genes, including activin receptor–like kinase 1 (ALK1). It remains unknown why and how selective blood vessels form AVMs, and there have been technical limitations to observing the initial stages of AVM formation. Here we present in vivo evidence that physiological or environmental factors such as wounds in addition to the genetic ablation are required for Alk1-deficient vessels to develop to AVMs in adult mice. Using the dorsal skinfold window chamber system, we have demonstrated for what we believe to be the first time the entire course of AVM formation in subdermal blood vessels by using intravital bright-field images, hyperspectral imaging, fluorescence recordings of direct arterial flow through the AV shunts, and vascular casting techniques. We believe our data provide novel insights into the pathogenetic mechanisms of HHT and potential therapeutic approaches.

Authors

Sung Ok Park, Mamta Wankhede, Young Jae Lee, Eun-Jung Choi, Naime Fliess, Se-Woon Choe, Seh-Hoon Oh, Glenn Walter, Mohan K. Raizada, Brian S. Sorg, S. Paul Oh

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The TGFβ superfamily in cardiac dysfunction
J Wu, O Jackson-Weaver, J Xu
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Consensus guidelines for the use and interpretation of angiogenesis assays
P Nowak-Sliwinska, K Alitalo, E Allen, A Anisimov, AC Aplin, R Auerbach, HG Augustin, DO Bates, JR van Beijnum, RH Bender, G Bergers, A Bikfalvi, J Bischoff, BC Böck, PC Brooks, F Bussolino, B Cakir, P Carmeliet, D Castranova, AM Cimpean, O Cleaver, G Coukos, GE Davis, MD Palma, A Dimberg, RP Dings, V Djonov, AC Dudley, NP Dufton, SM Fendt, N Ferrara, M Fruttiger, D Fukumura, B Ghesquière, Y Gong, RJ Griffin, AL Harris, CC Hughes, NW Hultgren, ML Iruela-Arispe, M Irving, RK Jain, R Kalluri, J Kalucka, RS Kerbel, J Kitajewski, I Klaassen, HK Kleinmann, P Koolwijk, E Kuczynski, BR Kwak, K Marien, JM Melero-Martin, LL Munn, RF Nicosia, A Noel, J Nurro, AK Olsson, TV Petrova, K Pietras, R Pili, JW Pollard, MJ Post, PH Quax, GA Rabinovich, M Raica, AM Randi, D Ribatti, C Ruegg, RO Schlingemann, S Schulte-Merker, LE Smith, JW Song, SA Stacker, J Stalin, AN Stratman, MV de Velde, VW van Hinsbergh, PB Vermeulen, J Waltenberger, BM Weinstein, H Xin, B Yetkin-Arik, S Yla-Herttuala, MC Yoder, AW Griffioen
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KJ Morine, X Qiao, V Paruchuri, MJ Aronovitz, EE Mackey, L Buiten, J Levine, K Ughreja, P Nepali, RM Blanton, SP Oh, RH Karas, NK Kapur
Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology 2017
Selective effects of oral antiangiogenic tyrosine kinase inhibitors on an animal model of hereditary hemorrhagic telangiectasia
YH Kim, MJ Kim, SW Choe, D Sprecher, YJ Lee, SP Oh
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JR Kraehling, JH Chidlow, C Rajagopal, MG Sugiyama, JW Fowler, MY Lee, X Zhang, CM Ramírez, EJ Park, B Tao, K Chen, L Kuruvilla, B Larriveé, E Folta-Stogniew, R Ola, N Rotllan, W Zhou, MW Nagle, J Herz, KJ Williams, A Eichmann, WL Lee, C Fernández-Hernando, WC Sessa
Nature Communications 2016
Defective fluid shear stress mechanotransduction mediates hereditary hemorrhagic telangiectasia
N Baeyens, B Larrivée, R Ola, B Hayward-Piatkowskyi, A Dubrac, B Huang, TD Ross, BG Coon, E Min, M Tsarfati, H Tong, A Eichmann, MA Schwartz
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Blood flow boosts BMP signaling to keep vessels in shape
CA Franco, H Gerhardt
The Journal of Cell Biology 2016
Alk1 controls arterial endothelial cell migration in lumenized vessels
ER Rochon, PG Menon, BL Roman
Development (Cambridge, England) 2016
Endothelial notch signaling is essential to prevent hepatic vascular malformations in mice: Notch Signaling Prevents Hepatic Vascular Malformations in Mice
H Cuervo, CM Nielsen, DA Simonetto, L Ferrell, VH Shah, RA Wang
Hepatology 2016
The pathobiology of vascular malformations: insights from human and model organism genetics: Genetics of vascular malformations
SE Wetzel-Strong, MR Detter, DA Marchuk
The Journal of Pathology 2016
PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia
R Ola, A Dubrac, J Han, F Zhang, JS Fang, B Larrivée, M Lee, AA Urarte, JR Kraehling, G Genet, KK Hirschi, WC Sessa, FV Canals, M Graupera, M Yan, LH Young, PS Oh, A Eichmann
Nature Communications 2016
A mouse model of hereditary hemorrhagic telangiectasia generated by transmammary-delivered immunoblocking of BMP9 and BMP10
S Ruiz, H Zhao, P Chandakkar, PK Chatterjee, J Papoin, L Blanc, CN Metz, F Campagne, P Marambaud
Scientific Reports 2016
Inhibition of ALK1 signaling with dalantercept combined with VEGFR TKI leads to tumor stasis in renal cell carcinoma
X Wang, N Solban, P Khanna, M Callea, J Song, DC Alsop, RS Pearsall, MB Atkins, JW Mier, S Signoretti, M Alimzhanov, R Kumar, MK Bhasin, RS Bhatt
Oncotarget 2016
Integrin β8 Deletion Enhances Vascular Dysplasia and Hemorrhage in the Brain of Adult Alk1 Heterozygous Mice
L Ma, F Shen, K Jun, C Bao, R Kuo, WL Young, SL Nishimura, H Su
Translational Stroke Research 2016
Matrix Gla protein limits pulmonary arteriovenous malformations in ALK1 deficiency
KI Bostrom, P Guihard, AM Medela, J Yao, JH Moon, A Penton, Y Yao
The European respiratory journal : official journal of the European Society for Clinical Respiratory Physiology 2015
Imaging of Small Animal Peripheral Artery Disease Models: Recent Advancements and Translational Potential
J Lin, E Phillips, TA Riggins, G Sangha, S Chakraborty, J Lee, R Lycke, C Hernandez, A Soepriatna, B Thorne, A Yrineo, C Goergen
International journal of molecular sciences 2015
Combined deficiency of Notch1 and Notch3 causes pericyte dysfunction, models CADASIL, and results in arteriovenous malformations
NM Kofler, H Cuervo, MK Uh, A Murtomäki, J Kitajewski
Scientific Reports 2015
Targeting BMP signalling in cardiovascular disease and anaemia
NW Morrell, DB Bloch, P Dijke, MJ Goumans, A Hata, J Smith, PB Yu, KD Bloch
Nature Reviews Cardiology 2015
Emerging roles of BMP9 and BMP10 in hereditary hemorrhagic telangiectasia
E Tillet, S Bailly
Frontiers in Genetics 2015
Mouse models of hereditary hemorrhagic telangiectasia: recent advances and future challenges
S Tual-Chalot, SP Oh, HM Arthur
Frontiers in Genetics 2015
Genetic variation in the functional ENG allele inherited from the non-affected parent associates with presence of pulmonary arteriovenous malformation in hereditary hemorrhagic telangiectasia 1 (HHT1) and may influence expression of PTPN14
TG Letteboer, M Benzinou, CB Merrick, DA Quigley, K Zhau, IJ Kim, MD To, DM Jablons, JK van Amstel, CJ Westermann, S Giraud, S Dupuis-Girod, G Lesca, JH Berg, A Balmain, RJ Akhurst
Frontiers in Genetics 2015
Endoglin involvement in integrin-mediated cell adhesion as a putative pathogenic mechanism in hereditary hemorrhagic telangiectasia type 1 (HHT1)
E Rossi, JÃ Lopez-Novoa, C Bernabeu
Frontiers in Genetics 2015
Pericytes as targets in hereditary hemorrhagic telangiectasia
JÃ Thalgott, D Dos-Santos-Luis, F Lebrin
Frontiers in Genetics 2015
Anti-angiogenic therapeutic strategies in hereditary hemorrhagic telangiectasia
DS Ardelean, M Letarte
Frontiers in Genetics 2015
Mouse Hepatic Tumor Vascular Imaging by Experimental Selective Angiography
SK Kim, H Kim, GY Koh, DS Lim, DY Yu, MD Kim, MS Park, JS Lim, XY Guan
PloS one 2015
Animal Models in Studying Cerebral Arteriovenous Malformation
M Xu, H Xu, Z Qin
BioMed Research International 2015
Experimental Animal Models of Arteriovenous Malformation: A Review
J Raj, M Stoodley
Veterinary Sciences 2015
Mouse Models of Cerebral Arteriovenous Malformation
CM Nielsen, L Huang, PA Murphy, MT Lawton, RA Wang
Stroke; a journal of cerebral circulation 2015
Constitutively active Notch4 receptor elicits brain arteriovenous malformations through enlargement of capillary-like vessels
PA Murphy, TN Kim, L Huang, CM Nielsen, MT Lawton, RH Adams, CB Schaffer, RA Wang
Proceedings of the National Academy of Sciences 2014
VEGF neutralization can prevent and normalize arteriovenous malformations in an animal model for hereditary hemorrhagic telangiectasia 2
C Han, S Choe, YH Kim, AP Acharya, BG Keselowsky, BS Sorg, YJ Lee, SP Oh
Angiogenesis 2014
Brain Arteriovenous Malformation Modeling, Pathogenesis, and Novel Therapeutic Targets
W Chen, EJ Choi, CM McDougall, H Su
Translational Stroke Research 2014
Endothelial depletion of acvrl1 in mice leads to arteriovenous malformations associated with reduced endoglin expression
S Tual-Chalot, M Mahmoud, KR Allinson, RE Redgrave, Z Zhai, SP Oh, M Fruttiger, HM Arthur
PloS one 2014
Novel brain arteriovenous malformation mouse models for type 1 hereditary hemorrhagic telangiectasia
EJ Choi, W Chen, K Jun, HM Arthur, WL Young, H Su
PloS one 2014
Zebrafish models of cerebrovascular disease
BP Walcott, RT Peterson
Journal of Cerebral Blood Flow & Metabolism 2014
Limitations of the dorsal skinfold window chamber model in evaluating anti-angiogenic therapy during early phase of angiogenesis
NM Biel, JA Lee, BS Sorg, DW Siemann
Vascular cell 2014
Essential role for TMEM100 in vascular integrity but limited contributions to the pathogenesis of hereditary haemorrhagic telangiectasia
EH Moon, YS Kim, J Seo, S Lee, YJ Lee, SP Oh
Cardiovascular Research 2014
Human brain arteriovenous malformations express lymphatic-associated genes
LD Shoemaker, LF Fuentes, SM Santiago, BM Allen, DJ Cook, GK Steinberg, SD Chang
Annals of Clinical and Translational Neurology 2014
Methods in Molecular Biology
W Chen, WL Young, H Su
Methods in molecular biology (Clifton, N.J.) 2014
De Novo Cerebrovascular Malformation in the Adult Mouse After Endothelial Alk1 Deletion and Angiogenic Stimulation
W Chen, Z Sun, Z Han, K Jun, M Camus, M Wankhede, L Mao, T Arnold, WL Young, H Su
Stroke; a journal of cerebral circulation 2014
ALK5 and ALK1 Play Antagonistic Roles in Transforming Growth Factor  -Induced Podosome Formation in Aortic Endothelial Cells
F Curado, P Spuul, I Egana, P Rottiers, T Daubon, V Veillat, P Duhamel, A Leclercq, E Gontier, E Genot
Molecular and cellular biology 2014
Deletion of Rbpj from postnatal endothelium leads to abnormal arteriovenous shunting in mice
CM Nielsen, H Cuervo, VW Ding, Y Kong, EJ Huang, RA Wang
Development (Cambridge, England) 2014
Developmental and pathological angiogenesis in the central nervous system
M Vallon, J Chang, H Zhang, CJ Kuo
Cellular and Molecular Life Sciences 2014
Somatic uniparental isodisomy explains multifocality of glomuvenous malformations
M Amyere, V Aerts, P Brouillard, BA McIntyre, FP Duhoux, M Wassef, O Enjolras, JB Mulliken, O Devuyst, H Antoine-Poirel, LM Boon, M Vikkula
The American Journal of Human Genetics 2013
Bone morphogenetic protein-9 inhibits lymphatic vessel formation via activin receptor-like kinase 1 during development and cancer progression
Y Yoshimatsu, YG Lee, Y Akatsu, L Taguchi, HI Suzuki, SI Cunha, K Maruyama, Y Suzuki, T Yamazaki, A Katsura, SP Oh, TA Zimmers, SJ Lee, K Pietras, GY Koh, K Miyazono, T Watabe
Proceedings of the National Academy of Sciences 2013
On the versatility of von Willebrand factor
A Rauch, N Wohner, OD Christophe, CV Denis, S Susen, PJ Lenting
Mediterranean journal of hematology and infectious diseases 2013
Reducing Jagged 1 and 2 levels prevents cerebral arteriovenous malformations in matrix Gla protein deficiency
Y Yao, J Yao, M Radparvar, AM Blazquez-Medela, PJ Guihard, M Jumabay, KI Boström
Proceedings of the National Academy of Sciences 2013
Endothelial signaling and the molecular basis of arteriovenous malformation
D Atri, B Larrivée, A Eichmann, M Simons
Cellular and Molecular Life Sciences 2013
Directional next-generation RNA sequencing and examination of premature termination codon mutations in endoglin/hereditary haemorrhagic telangiectasia
FS Govani, A Giess, IG Mollet, ME Begbie, MD Jones, L Game, CL Shovlin
Molecular syndromology 2013
SnoN facilitates ALK1-Smad1/5 signaling during embryonic angiogenesis
Q Zhu, YH Kim, D Wang, SP Oh, K Luo
The Journal of Cell Biology 2013
Enhanced responses to angiogenic cues underlie the pathogenesis of hereditary hemorrhagic telangiectasia 2
EJ Choi, YH Kim, S Choe, YG Tak, EM Garrido-Martin, M Chang, YJ Lee, SP Oh
PloS one 2013
Vascular Injury Triggers Krüppel-Like Factor 6 Mobilization and Cooperation With Specificity Protein 1 to Promote Endothelial Activation Through Upregulation of the Activin Receptor-Like Kinase 1 Gene
EM Garrido-Martín, FJ Blanco, M Roquè, L Novensà, M Tarocchi, UE Lang, T Suzuki, SL Friedman, LM Botella, C Bernabéu
Circulation research 2013
Minimal homozygous endothelial deletion of Eng with VEGF stimulation is sufficient to cause cerebrovascular dysplasia in the adult mouse
EJ Choi, EJ Walker, F Shen, SP Oh, HM Arthur, WL Young, H Su
Cerebrovascular Diseases 2012
Signaling Required for Blood Vessel Maintenance: Molecular Basis and Pathological Manifestations
M Murakami
International Journal of Vascular Medicine 2012
Transforming growth factor-β and smooth muscle differentiation
X Guo, SY Chen
World journal of biological chemistry 2012
Vascular anomalies: from genetics toward models for therapeutic trials
M Uebelhoer, LM Boon, M Vikkula
Cold Spring Harbor Perspectives in Medicine 2012
Endothelial Expression of TGFβ Type II Receptor Is Required to Maintain Vascular Integrity during Postnatal Development of the Central Nervous System
KR Allinson, HS Lee, M Fruttiger, J McCarty, HM Arthur
PloS one 2012
Gastric angiodysplasia in a hereditary hemorrhagic telangiectasia type 2 patient
M Ha, YJ Kim, KA Kwon, KB Hahm, MJ Kim, DK Kim, YJ Lee, SP Oh
World journal of gastroenterology : WJG 2012
Reduced mural cell coverage and impaired vessel integrity after angiogenic stimulation in the Alk1-deficient brain
W Chen, Y Guo, EJ Walker, F Shen, K Jun, SP Oh, V Degos, MT Lawton, T Tihan, D Davalos, K Akassoglou, J Nelson, J Pile-Spellman, H Su, WL Young
Arteriosclerosis, thrombosis, and vascular biology 2012
BMP9 induces EphrinB2 expression in endothelial cells through an Alk1-BMPRII/ActRII-ID1/ID3-dependent pathway: implications for hereditary hemorrhagic telangiectasia type II
JH Kim, MR Peacock, SC George, CC Hughes
Angiogenesis 2012
Bevacizumab attenuates VEGF-induced angiogenesis and vascular malformations in the adult mouse brain
EJ Walker, H Su, F Shen, V Degos, G Amend, K Jun, WL Young
Stroke; a journal of cerebral circulation 2012
BMP9 and BMP10 are critical for postnatal retinal vascular remodeling
N Ricard, D Ciais, S Levet, M Subileau, C Mallet, TA Zimmers, SJ Lee, M Bidart, JJ Feige, S Bailly
Blood 2012
Anti-human activin receptor-like kinase 1 (ALK1) antibody attenuates bone morphogenetic protein 9 (BMP9)-induced ALK1 signaling and interferes with endothelial cell sprouting
LA van Meeteren, M Thorikay, S Bergqvist, E Pardali, CG Stampino, D Hu-Lowe, MJ Goumans, P Dijke
The Journal of biological chemistry 2012
ALK1 signaling inhibits angiogenesis by cooperating with the Notch pathway
B Larrivée, C Prahst, E Gordon, R Toro, T Mathivet, A Duarte, M Simons, A Eichmann
Developmental Cell 2012
Mouse and human strategies identify PTPN14 as a modifier of angiogenesis and hereditary haemorrhagic telangiectasia
M Benzinou, FF Clermont, TG Letteboer, JH Kim, S Espejel, KA Harradine, J Arbelaez, MT Luu, R Roy, D Quigley, MN Higgins, M Zaid, BE Aouizerat, JK van Amstel, S Giraud, S Dupuis-Girod, G Lesca, H Plauchu, CC Hughes, CJ Westermann, RJ Akhurst
Nature Communications 2012
Arteriovenous Malformations and Other Vascular Malformation Syndromes
KJ Whitehead, MC Smith, DY Li
Cold Spring Harbor Perspectives in Medicine 2012
Arteriovenous Malformation in the Adult Mouse Brain Resembling the Human Disease
EJ Walker, H Su, F Shen, EJ Choi, SP Oh, G Chen, MT Lawton, H Kim, Y Chen, W Chen, WL Young
Annals of Neurology 2011
Interaction between alk1 and blood flow in the development of arteriovenous malformations
P Corti, S Young, CY Chen, MJ Patrick, ER Rochon, K Pekkan, BL Roman
Development (Cambridge, England) 2011
Surgical Approaches to Create Murine Models of Human Wound Healing
VW Wong, M Sorkin, JP Glotzbach, MT Longaker, GC Gurtner
Journal of Biomedicine and Biotechnology 2011
Cerebrovascular disorders: molecular insights and therapeutic opportunities
E Storkebaum, A Quaegebeur, M Vikkula, P Carmeliet
Nature Neuroscience 2011
ALK1 as an emerging target for antiangiogenic therapy of cancer
SI Cunha, K Pietras
Blood 2011
Brain Arteriovenous Malformation Pathogenesis: A Response-to-Injury Paradigm
H Kim, H Su, S Weinsheimer, L Pawlikowska, WL Young
Acta neurochirurgica. Supplement 2011
Brain arteriovenous malformation multiplicity predicts the diagnosis of hereditary hemorrhagic telangiectasia: quantitative assessment
A Bharatha, ME Faughnan, H Kim, T Pourmohamad, T Krings, P Bayrak-Toydemir, L Pawlikowska, CE McCulloch, MT Lawton, CF Dowd, WL Young, KG Terbrugge
Stroke; a journal of cerebral circulation 2011
Endothelial von Willebrand factor regulates angiogenesis
RD Starke, F Ferraro, KE Paschalaki, NH Dryden, TA McKinnon, RE Sutton, EM Payne, DO Haskard, AD Hughes, DF Cutler, MA Laffan, AM Randi
Blood 2010
BMP signaling in vascular development and disease
JW Lowery, MP de Caestecker
Cytokine & Growth Factor Reviews 2010
Fine mapping of the hereditary haemorrhagic telangiectasia (HHT)3 locus on chromosome 5 excludes VE-Cadherin-2, Sprouty4 and other interval genes
FS Govani, CL Shovlin
Journal of angiogenesis research 2010
VEGF Induces More Severe Cerebrovascular Dysplasia in Eng+/− than in Alk1+/− Mice
Q Hao, Y Zhu, H Su, F Shen, GY Yang, H Kim, WL Young
Translational Stroke Research 2010
Spectral imaging reveals microvessel physiology and function from anastomoses to thromboses
M Wankhede, N Agarwal, RA Fraga-Silva, C deDeugd, MK Raizada, SP Oh, BS Sorg
Journal of Biomedical Optics 2010
Reply to Fernández-Fernández
Shovlin CL, Govani FS
European Journal of Human Genetics 2009

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