Acvrl1

Summary

Gene Symbol: Acvrl1
Description: activin A receptor, type II-like 1
Alias: AI115505, AI427544, Acvrlk1, Alk1, serine/threonine-protein kinase receptor R3, SKR3, TGF-B superfamily receptor type I, TSR-I, activin receptor-like kinase 1
Species: mouse
Products:     Acvrl1

Top Publications

  1. Oh S, Seki T, Goss K, Imamura T, Yi Y, Donahoe P, et al. Activin receptor-like kinase 1 modulates transforming growth factor-beta 1 signaling in the regulation of angiogenesis. Proc Natl Acad Sci U S A. 2000;97:2626-31 pubmed
    The activin receptor-like kinase 1 (ALK1) is a type I receptor for transforming growth factor-beta (TGF-beta) family proteins...
  2. Goumans M, Valdimarsdottir G, Itoh S, Lebrin F, Larsson J, Mummery C, et al. Activin receptor-like kinase (ALK)1 is an antagonistic mediator of lateral TGFbeta/ALK5 signaling. Mol Cell. 2003;12:817-28 pubmed
    ..Activin receptor-like kinase-1 (ALK1) induces Smad1/5 phosphorylation, leading to an increase in endothelial cell proliferation and migration, while ..
  3. 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
    ..b>ALK1 is a member of the transforming growth factor-beta type I family of receptors, and compelling genetic evidence ..
  4. Cunha S, Pardali E, Thorikay M, Anderberg C, Hawinkels L, Goumans M, et al. Genetic and pharmacological targeting of activin receptor-like kinase 1 impairs tumor growth and angiogenesis. J Exp Med. 2010;207:85-100 pubmed publisher
    ..Diminution of ALK1 gene dosage or systemic treatment with the ALK1-Fc fusion protein RAP-041 retarded tumor growth and progression by ..
  5. Chen W, Sun Z, Han Z, Jun K, Camus M, Wankhede M, et al. De novo cerebrovascular malformation in the adult mouse after endothelial Alk1 deletion and angiogenic stimulation. Stroke. 2014;45:900-2 pubmed publisher
    In humans, activin receptor-like kinase 1 (Alk1) deficiency causes arteriovenous malformations (AVMs) in multiple organs, including the brain...
  6. Chen W, Guo Y, Walker E, Shen F, Jun K, Oh S, et al. Reduced mural cell coverage and impaired vessel integrity after angiogenic stimulation in the Alk1-deficient brain. Arterioscler Thromb Vasc Biol. 2013;33:305-10 pubmed publisher
    ..Hereditary hemorrhagic telangiectasia type 2 patients with activin receptor-like kinase 1 (Alk1) mutation have a higher incidence of brain arteriovenous malformation than the general population...
  7. Roelen B, Van Rooijen M, Mummery C. Expression of ALK-1, a type 1 serine/threonine kinase receptor, coincides with sites of vasculogenesis and angiogenesis in early mouse development. Dev Dyn. 1997;209:418-30 pubmed
    ..Its expression pattern suggests that ALK-1 is a type I receptor for TGF-beta1 in the developing mouse. ..
  8. Park S, Lee Y, Seki T, Hong K, Fliess N, Jiang Z, et al. ALK5- and TGFBR2-independent role of ALK1 in the pathogenesis of hereditary hemorrhagic telangiectasia type 2. Blood. 2008;111:633-42 pubmed
    b>ALK1 belongs to the type I receptor family for transforming growth factor-beta family ligands. Heterozygous ALK1 mutations cause hereditary hemorrhagic telangiectasia type 2 (HHT2), a multisystemic vascular disorder...
  9. Garrido Martin E, Blanco F, Roque M, Novensa L, Tarocchi M, Lang U, et al. 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. Circ Res. 2013;112:113-27 pubmed publisher
    Activin receptor-like kinase-1 (ALK1) is an endothelial transforming growth factor ? receptor involved in angiogenesis...

More Information

Publications70

  1. Seki T, Yun J, Oh S. Arterial endothelium-specific activin receptor-like kinase 1 expression suggests its role in arterialization and vascular remodeling. Circ Res. 2003;93:682-9 pubmed
    ..Two genes are linked to HHT: endoglin (ENG) in HHT1 and activin receptor-like kinase 1 (ACVRL1; ALK1) in HHT2...
  2. Suzuki Y, Ohga N, Morishita Y, Hida K, Miyazono K, Watabe T. BMP-9 induces proliferation of multiple types of endothelial cells in vitro and in vivo. J Cell Sci. 2010;123:1684-92 pubmed publisher
    ..These findings suggest that BMP-9 signaling activates the endothelium tested in the present study via ALK-1. ..
  3. Mahmoud M, Borthwick G, Hislop A, Arthur H. Endoglin and activin receptor-like-kinase 1 are co-expressed in the distal vessels of the lung: implications for two familial vascular dysplasias, HHT and PAH. Lab Invest. 2009;89:15-25 pubmed publisher
    ..in transforming growth factor-beta family signalling, either endoglin (ENG) or activin receptor-like kinase 1 (ACVRL1, also known as ALK1)...
  4. Jerkic M, Kabir M, Davies A, Yu L, McIntyre B, Husain N, et al. Pulmonary hypertension in adult Alk1 heterozygous mice due to oxidative stress. Cardiovasc Res. 2011;92:375-84 pubmed publisher
    Mutations in the ALK1 gene, coding for an endothelial-specific receptor of the transforming growth factor-? superfamily, are the underlying cause of hereditary haemorrhagic telangiectasia type 2, but are also associated with familial ..
  5. Roman B, Pham V, Lawson N, Kulik M, Childs S, Lekven A, et al. Disruption of acvrl1 increases endothelial cell number in zebrafish cranial vessels. Development. 2002;129:3009-19 pubmed
    ..We show that vbg encodes activin receptor-like kinase 1 (Acvrl1; also known as Alk1), a TGFbeta type I receptor that is expressed predominantly in the endothelium of the vessels ..
  6. Mitchell D, Pobre E, Mulivor A, Grinberg A, Castonguay R, Monnell T, et al. ALK1-Fc inhibits multiple mediators of angiogenesis and suppresses tumor growth. Mol Cancer Ther. 2010;9:379-88 pubmed publisher
    Activin receptor-like kinase-1 (ALK1) is a type I, endothelial cell-specific member of the transforming growth factor-beta superfamily of receptors known to play an essential role in modulating angiogenesis and vessel maintenance...
  7. Urness L, Sorensen L, Li D. Arteriovenous malformations in mice lacking activin receptor-like kinase-1. Nat Genet. 2000;26:328-31 pubmed
    ..that HHT can be caused by loss-of-function mutations in the gene encoding activin receptor-like kinase-1 (ACVRL1; ref. 5). ACVRL1 encodes a type I receptor for the TGF-beta superfamily of growth factors...
  8. Park S, Wankhede M, Lee Y, Choi E, Fliess N, Choe S, et al. Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia. J Clin Invest. 2009;119:3487-96 pubmed publisher
    ..and visceral AVMs and caused by mutations in one of several genes, including activin receptor-like kinase 1 (ALK1)...
  9. Muñoz Félix J, Lopez Novoa J, Martinez Salgado C. Heterozygous disruption of activin receptor-like kinase 1 is associated with increased renal fibrosis in a mouse model of obstructive nephropathy. Kidney Int. 2014;85:319-32 pubmed publisher
    ..Activin receptor-like kinase 1 (ALK1) is a transforming growth factor-?1 type I receptor with a pivotal role in endothelial proliferation and migration, ..
  10. Hao Q, Su H, Marchuk D, Rola R, Wang Y, Liu W, et al. Increased tissue perfusion promotes capillary dysplasia in the ALK1-deficient mouse brain following VEGF stimulation. Am J Physiol Heart Circ Physiol. 2008;295:H2250-6 pubmed publisher
    Loss-of-function activin receptor-like kinase 1 gene mutation (ALK1+/-) is associated with brain arteriovenous malformations (AVM) in hereditary hemorrhagic telangiectasia type 2. Other determinants of the lesional phenotype are unknown...
  11. Brown M, Zhao Q, Baker K, Naik C, Chen C, Pukac L, et al. Crystal structure of BMP-9 and functional interactions with pro-region and receptors. J Biol Chem. 2005;280:25111-8 pubmed
    ..pro-region complex in cell-based assays. ..
  12. Larrivee B, Prahst C, Gordon E, del Toro R, Mathivet T, Duarte A, et al. ALK1 signaling inhibits angiogenesis by cooperating with the Notch pathway. Dev Cell. 2012;22:489-500 pubmed publisher
    Activin receptor-like kinase 1 (ALK1) is an endothelial-specific member of the TGF-?/BMP receptor family that is inactivated in patients with hereditary hemorrhagic telangiectasia (HHT)...
  13. Srinivasan S, Hanes M, Dickens T, Porteous M, Oh S, Hale L, et al. A mouse model for hereditary hemorrhagic telangiectasia (HHT) type 2. Hum Mol Genet. 2003;12:473-82 pubmed
    ..HHT type 2 is caused by loss of function mutations in activin receptor-like kinase 1 (ACVRL1 or ALK1)...
  14. Curado F, Spuul P, Egaña I, Rottiers P, Daubon T, Veillat V, et al. ALK5 and ALK1 play antagonistic roles in transforming growth factor β-induced podosome formation in aortic endothelial cells. Mol Cell Biol. 2014;34:4389-403 pubmed publisher
    ..To explore further this TGF-β-specific response and determine in which context podosomes form, ALK5 and ALK1 TGF-β receptor signaling pathways were investigated in bovine aortic endothelial cells...
  15. Kraehling J, Chidlow J, Rajagopal C, Sugiyama M, Fowler J, Lee M, et al. Genome-wide RNAi screen reveals ALK1 mediates LDL uptake and transcytosis in endothelial cells. Nat Commun. 2016;7:13516 pubmed publisher
    ..Here we show that the activin-like kinase 1 (ALK1) mediates LDL uptake into endothelial cells...
  16. Aspalter I, Gordon E, Dubrac A, Ragab A, Narloch J, Vizán P, et al. Alk1 and Alk5 inhibition by Nrp1 controls vascular sprouting downstream of Notch. Nat Commun. 2015;6:7264 pubmed publisher
    ..function of neuropilin-1 (Nrp1), which suppresses the stalk-cell phenotype by limiting Smad2/3 activation through Alk1 and Alk5...
  17. Seki T, Hong K, Oh S. Nonoverlapping expression patterns of ALK1 and ALK5 reveal distinct roles of each receptor in vascular development. Lab Invest. 2006;86:116-29 pubmed
    ..We have previously shown that another type I receptor, ALK1 (ACVRL1), can also mediate TGF-beta signals via BMP-activated Smads in vascular endothelial cells (ECs)...
  18. Park K, Morrison C, Sorensen L, Jones C, Rao Y, Chien C, et al. Robo4 is a vascular-specific receptor that inhibits endothelial migration. Dev Biol. 2003;261:251-67 pubmed
    ..These data demonstrate that Robo4 is a bona fide member of the Robo family and may provide a repulsive cue to migrating endothelial cells during vascular development. ..
  19. Alejandre Alcazar M, Michiels Corsten M, Vicencio A, Reiss I, Ryu J, de Krijger R, et al. TGF-beta signaling is dynamically regulated during the alveolarization of rodent and human lungs. Dev Dyn. 2008;237:259-69 pubmed
    ..Pronounced changes in the expression and localization of the TGF-beta receptors Acvrl1, Tgfbr1, Tgfbr2, Tgfbr3, and endoglin, and the intracellular messengers Smad2, Smad3, Smad4, Smad6, and Smad7 were ..
  20. Tulachan S, Tei E, Hembree M, Crisera C, Prasadan K, Koizumi M, et al. TGF-beta isoform signaling regulates secondary transition and mesenchymal-induced endocrine development in the embryonic mouse pancreas. Dev Biol. 2007;305:508-21 pubmed
    ..The TGF-beta isoform receptors (RI, RII and ALK1) were localized mainly to both the pancreatic epithelium and mesenchyme at early stages of development, but then ..
  21. Yao Y, Nowak S, Yochelis A, Garfinkel A, BOSTROM K. Matrix GLA protein, an inhibitory morphogen in pulmonary vascular development. J Biol Chem. 2007;282:30131-42 pubmed
  22. Yao Y, Jumabay M, Wang A, BOSTROM K. Matrix Gla protein deficiency causes arteriovenous malformations in mice. J Clin Invest. 2011;121:2993-3004 pubmed publisher
    ..of hereditary hemorrhagic telangiectasia (HHT), a disease caused by mutations in activin-like kinase receptor 1 (ALK1), which is an essential receptor in angiogenesis, or endoglin...
  23. Li H, Zhao D, Wang S, Ding J, Zhao L. Bone morphogenetic protein?9 promotes the differentiation of mouse spleen macrophages into osteoclasts via the ALK1 receptor and ERK 1/2 pathways in vitro. Mol Med Rep. 2016;14:4545-4550 pubmed publisher
    ..The expression levels of cell BMP receptor?IA (BMPR?IA), BMPR?IB, BMPR?II and anaplastic lymphoma kinase 1 (ALK1) receptor were detected by ELISA, the small mothers against decapentaplegic pathway, extracellular signal?regulated ..
  24. Arnold T, Niaudet C, Pang M, Siegenthaler J, Gaengel K, Jung B, et al. Excessive vascular sprouting underlies cerebral hemorrhage in mice lacking αVβ8-TGFβ signaling in the brain. Development. 2014;141:4489-99 pubmed publisher
    ..Our data therefore suggest that abnormal vascular sprouting and patterning, not BBB dysfunction, underlie developmental cerebral hemorrhage. ..
  25. Kim J, Kim M, Jeong Y, Lee W, Park H, Kwon J, et al. BMP9 Induces Cord Blood-Derived Endothelial Progenitor Cell Differentiation and Ischemic Neovascularization via ALK1. Arterioscler Thromb Vasc Biol. 2015;35:2020-31 pubmed publisher
    ..The expression levels of activin receptor-like kinase 1 (ALK1) and its high-affinity ligand, bone morphogenetic protein 9 (BMP9) were markedly changed in EPC→endothelial ..
  26. Pece Barbara N, Vera S, Kathirkamathamby K, Liebner S, Di Guglielmo G, Dejana E, et al. Endoglin null endothelial cells proliferate faster and are more responsive to transforming growth factor beta1 with higher affinity receptors and an activated Alk1 pathway. J Biol Chem. 2005;280:27800-8 pubmed
    ..Moreover, mutations in endoglin and the endothelial-specific TGFbeta type I receptor, ALK1, are linked to hereditary hemorrhagic telangiectasia...
  27. Muñoz Félix J, Perretta Tejedor N, Eleno N, Lopez Novoa J, Martinez Salgado C. ALK1 heterozygosity increases extracellular matrix protein expression, proliferation and migration in fibroblasts. Biochim Biophys Acta. 2014;1843:1111-22 pubmed publisher
    ..Transforming growth factor beta 1 (TGF-?1) plays a major role in tissue fibrosis. Activin receptor-like kinase 1 (ALK1) is a type I receptor of TGF-?1 with an important role in angiogenesis whose function in cellular biology and TGF-? ..
  28. Ola R, Dubrac A, Han J, Zhang F, Fang J, Larrivee B, et al. PI3 kinase inhibition improves vascular malformations in mouse models of hereditary haemorrhagic telangiectasia. Nat Commun. 2016;7:13650 pubmed publisher
    Activin receptor-like kinase 1 (ALK1) is an endothelial serine-threonine kinase receptor for bone morphogenetic proteins (BMPs) 9 and 10...
  29. Dewulf N, Verschueren K, Lonnoy O, Moren A, Grimsby S, Vande Spiegle K, et al. Distinct spatial and temporal expression patterns of two type I receptors for bone morphogenetic proteins during mouse embryogenesis. Endocrinology. 1995;136:2652-63 pubmed
    ..In addition, the expression of these genes in many soft tissues suggests broader functions for BMPs in embryogenesis. ..
  30. Verma R, Jaiswal H, Chauhan K, Kaushik M, Tailor P. Cutting Edge: ACVRL1 Signaling Augments CD8?+ Dendritic Cell Development. J Immunol. 2016;197:1029-34 pubmed publisher
    ..We analyzed the effect of Irf8 on TGF-?/bone morphogenetic protein pathway-specific genes in DCs and identified Acvrl1, a type I TGF-? superfamily receptor, as a gene strongly induced by Irf8 expression...
  31. Song T, Wang W, Xu J, Zhao D, Dong Q, Li L, et al. Fibroblast growth factor 2 inhibits bone morphogenetic protein 9-induced osteogenic differentiation of mesenchymal stem cells by repressing Smads signaling and subsequently reducing Smads dependent up-regulation of ALK1 and ALK2. Int J Biochem Cell Biol. 2013;45:1639-46 pubmed publisher
    ..differentiation by blocking BMP9-induced Smads signaling and subsequently reducing Smads dependent up-regulation of ALK1 and ALK2 in MSCs...
  32. Garrido Martin E, Nguyen H, Cunningham T, Choe S, Jiang Z, Arthur H, et al. Common and distinctive pathogenetic features of arteriovenous malformations in hereditary hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 animal models--brief report. Arterioscler Thromb Vasc Biol. 2014;34:2232-6 pubmed publisher
    ..hemorrhagic telangiectasia 1 and hereditary hemorrhagic telangiectasia 2 are caused by mutations in ENG and ALK1, respectively...
  33. Takahashi T, Takahashi K, St John P, Fleming P, Tomemori T, Watanabe T, et al. A mutant receptor tyrosine phosphatase, CD148, causes defects in vascular development. Mol Cell Biol. 2003;23:1817-31 pubmed
    ..These findings implicate a member of the receptor tyrosine phosphatase family, CD148, in developmental vascular organization and provide evidence that it regulates endothelial proliferation and endothelium-pericyte interactions. ..
  34. Morine K, Qiao X, Paruchuri V, Aronovitz M, Mackey E, Buiten L, et al. Conditional knockout of activin like kinase-1 (ALK-1) leads to heart failure without maladaptive remodeling. Heart Vessels. 2017;32:628-636 pubmed publisher
    ..Future studies exploring the functional role of AlK-1 in cardiac remodeling independent of systemic AVMs are required. ..
  35. Ma L, Shen F, Jun K, Bao C, Kuo R, Young W, et al. Integrin ?8 Deletion Enhances Vascular Dysplasia and Hemorrhage in the Brain of Adult Alk1 Heterozygous Mice. Transl Stroke Res. 2016;7:488-496 pubmed
    ..Patients with haploinsufficiency of transforming growth factor-? (TGF-?) receptors, activin receptor-like kinase 1 (ALK1) or endoglin (ENG) have a higher incidence of bAVM than the general population...
  36. Dudas M, Nagy A, Laping N, Moustakas A, Kaartinen V. Tgf-beta3-induced palatal fusion is mediated by Alk-5/Smad pathway. Dev Biol. 2004;266:96-108 pubmed
    ..Based on these observations, we conclude that the Smad2-dependent Alk-5 signaling pathway is dominant in palatal fusion driven by Tgf-beta3. ..
  37. Mallet C, Vittet D, Feige J, Bailly S. TGFbeta1 induces vasculogenesis and inhibits angiogenic sprouting in an embryonic stem cell differentiation model: respective contribution of ALK1 and ALK5. Stem Cells. 2006;24:2420-7 pubmed
    ..present study was to investigate the role of TGFbeta1 and its two type I receptors, activin receptor-like kinase-1 (ALK1) and ALK5, in an embryonic stem cell (ESC) differentiation model that recapitulates the developmental steps of ..
  38. Hong K, Seki T, Oh S. Activin receptor-like kinase 1 is essential for placental vascular development in mice. Lab Invest. 2007;87:670-9 pubmed
    Activin receptor-like kinase 1 (ALK1) is involved in the pathogenesis of hereditary hemorrhagic telangiectasia type II (HHT2) and pulmonary arterial hypertension...
  39. 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
  40. Wu X, Robinson C, Fong H, Crabtree J, Rodriguez B, Roe B, et al. Cloning and characterization of the murine activin receptor like kinase-1 (ALK-1) homolog. Biochem Biophys Res Commun. 1995;216:78-83 pubmed
    ..Two protein species of 60 kDa and 72-76 kDa were detected. Glycosylation events or alternative splicing may account for the larger protein specie. ..
  41. Tual Chalot S, Mahmoud M, Allinson K, Redgrave R, Zhai Z, Oh S, et al. Endothelial depletion of Acvrl1 in mice leads to arteriovenous malformations associated with reduced endoglin expression. PLoS ONE. 2014;9:e98646 pubmed publisher
    ..The majority of patients carry mutations in either Endoglin (ENG) or ACVRL1 (also known as ALK1) genes, and the disease is characterized by arteriovenous malformations and persistent ..
  42. Korff T, Aufgebauer K, Hecker M. Cyclic stretch controls the expression of CD40 in endothelial cells by changing their transforming growth factor-beta1 response. Circulation. 2007;116:2288-97 pubmed
    ..This mechanism in turn may be responsible for the heterogeneous expression of CD40 at arterial bifurcations or curvatures and would support a site-specific proinflammatory response that is typical for the early phase of atherosclerosis. ..
  43. 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
  44. Albe E, Escalona E, Rajagopal R, Javier J, Chang J, Azar D. Proteomic identification of activin receptor-like kinase-1 as a differentially expressed protein during hyaloid vascular system regression. FEBS Lett. 2005;579:5481-6 pubmed
    ..We used proteomic analysis and immunohistochemical staining to identify activin receptor-like kinase-1 (ALK1), a type I receptor for transforming growth factor-beta1, during the HVS regression phase...
  45. Yao Y, Jumabay M, Ly A, Radparvar M, Wang A, Abdmaulen R, et al. Crossveinless 2 regulates bone morphogenetic protein 9 in human and mouse vascular endothelium. Blood. 2012;119:5037-47 pubmed publisher
    ..by matrix Gla protein (MGP) and crossveinless 2 (CV2), both induced by the activin receptor like-kinase 1 (ALK1) when stimulated by BMP-9...
  46. Seghers L, De Vries M, Pardali E, Hoefer I, Hierck B, Ten Dijke P, et al. Shear induced collateral artery growth modulated by endoglin but not by ALK1. J Cell Mol Med. 2012;16:2440-50 pubmed publisher
    ..Unilateral femoral artery ligation was performed in mice heterozygous for either endoglin or ALK1 and in littermate controls...
  47. Seki T, Hong K, Yun J, Kim S, Oh S. Isolation of a regulatory region of activin receptor-like kinase 1 gene sufficient for arterial endothelium-specific expression. Circ Res. 2004;94:e72-7 pubmed
    Activin receptor-like kinase 1 (Acvrl1; Alk1) is a type I receptor for transforming growth factor-beta (TGF-beta)...
  48. González Núñez M, Riolobos A, Castellano O, Fuentes Calvo I, de los Ángeles Sevilla M, Oujo B, et al. Heterozygous disruption of activin receptor-like kinase 1 is associated with increased arterial pressure in mice. Dis Model Mech. 2015;8:1427-39 pubmed publisher
    ..The purpose of this study was to assess the role of ALK-1 in regulation of AP using Alk1 haploinsufficient mice (Alk1(+/-))...
  49. Gkatzis K, Thalgott J, Dos Santos Luis D, Martin S, Lamandé N, Carette M, et al. Interaction Between ALK1 Signaling and Connexin40 in the Development of Arteriovenous Malformations. Arterioscler Thromb Vasc Biol. 2016;36:707-17 pubmed publisher
    ..We next took advantage of the Acvrl1(+/-) mutant mice that develop lesions similar to those in patients with HHT2 and generated Acvrl1(+/-); Gja5(EGFP/+..
  50. Choi E, Walker E, Shen F, Oh S, Arthur H, Young W, et al. Minimal homozygous endothelial deletion of Eng with VEGF stimulation is sufficient to cause cerebrovascular dysplasia in the adult mouse. Cerebrovasc Dis. 2012;33:540-7 pubmed publisher
    ..Telangiectasia (HHT) patients with haploinsufficiency of endoglin (ENG, HHT1) or activin receptor-like kinase 1 (ALK1, HHT2) have a higher incidence of bAVM than the general population...
  51. Blaney Davidson E, Remst D, Vitters E, van Beuningen H, Blom A, Goumans M, et al. Increase in ALK1/ALK5 ratio as a cause for elevated MMP-13 expression in osteoarthritis in humans and mice. J Immunol. 2009;182:7937-45 pubmed publisher
    ..it was shown that besides signaling via the ALK5 (activin-like kinase 5) receptor, TGF-beta can also signal via ALK1, thereby activating Smad1/5/8 instead of Smad2/3...
  52. Scharpfenecker M, Floot B, Korlaar R, Russell N, Stewart F. ALK1 heterozygosity delays development of late normal tissue damage in the irradiated mouse kidney. Radiother Oncol. 2011;99:349-55 pubmed publisher
    Activin receptor-like kinase 1 (ALK1) is a transforming growth factor ? (TGF-?) receptor, which is mainly expressed in endothelial cells regulating proliferation and migration in vitro and angiogenesis in vivo...
  53. Sorensen L, Brooke B, Li D, Urness L. Loss of distinct arterial and venous boundaries in mice lacking endoglin, a vascular-specific TGFbeta coreceptor. Dev Biol. 2003;261:235-50 pubmed
    ..The genes implicated in this disease, ALK1 and endoglin, may be involved in defining the fundamental boundaries between arteries and veins...
  54. Gore B, Izikki M, Mercier O, Dewachter L, Fadel E, Humbert M, et al. Key role of the endothelial TGF-?/ALK1/endoglin signaling pathway in humans and rodents pulmonary hypertension. PLoS ONE. 2014;9:e100310 pubmed publisher
    ..To determine whether the TGF-?/ALK1/ENG pathway was involved in PAH, we investigated pulmonary TGF-?, ALK1, ALK5, and ENG expressions in human lung ..
  55. Luo J, Tang M, Huang J, He B, Gao J, Chen L, et al. TGFbeta/BMP type I receptors ALK1 and ALK2 are essential for BMP9-induced osteogenic signaling in mesenchymal stem cells. J Biol Chem. 2010;285:29588-98 pubmed publisher
    ..BMP9 is one of the least studied BMPs. Whereas ALK1, ALK5, and/or endoglin have recently been reported as potential BMP9 type I receptors in endothelial cells, little ..
  56. Eleftheriou N, Sjölund J, Bocci M, Cortez E, Lee S, Cunha S, et al. Compound genetically engineered mouse models of cancer reveal dual targeting of ALK1 and endoglin as a synergistic opportunity to impinge on angiogenic TGF-β signaling. Oncotarget. 2016;7:84314-84325 pubmed publisher
    ..Here we investigate the interaction of two such receptors, ALK1 and endoglin, in pancreatic neuroendocrine tumors (PanNET)...
  57. Vorstenbosch J, Nguyen C, Zhou S, Seo Y, Siblini A, Finnson K, et al. Overexpression of CD109 in the Epidermis Differentially Regulates ALK1 Versus ALK5 Signaling and Modulates Extracellular Matrix Synthesis in the Skin. J Invest Dermatol. 2017;137:641-649 pubmed publisher
    ..to the canonical type I receptor, ALK5, which activates Smad2/3, TGF-? can signal through another type I receptor, ALK1, which activates Smad1/5...
  58. Shao E, Lin L, Yao Y, BOSTROM K. Expression of vascular endothelial growth factor is coordinately regulated by the activin-like kinase receptors 1 and 5 in endothelial cells. Blood. 2009;114:2197-206 pubmed publisher
    ..The objective was to examine regulation of VEGF by the activin-like kinase receptors (ALKs) ALK1 and ALK5...
  59. Shahid M, Spagnolli E, Ernande L, Thoonen R, Kolodziej S, Leyton P, et al. BMP type I receptor ALK2 is required for angiotensin II-induced cardiac hypertrophy. Am J Physiol Heart Circ Physiol. 2016;310:H984-94 pubmed publisher
    ..Cardiomyocyte-specific deletion of BMP type I receptor ALK2 (activin-like kinase 2), but not ALK1 or ALK3, inhibited BMP signaling and mitigated A2-induced cardiac hypertrophy and left ventricular fibrosis in mice...
  60. Wang X, Abraham S, McKenzie J, Jeffs N, Swire M, Tripathi V, et al. LRG1 promotes angiogenesis by modulating endothelial TGF-β signalling. Nature. 2013;499:306-11 pubmed publisher
    ..LRG1 antibody blockade inhibits this switch and attenuates angiogenesis. These studies reveal a new regulator of angiogenesis that mediates its effect by modulating TGF-β signalling. ..
  61. Mizuta K, Sakabe M, Hashimoto A, Ioka T, Sakai C, Okumura K, et al. Impairment of endothelial-mesenchymal transformation during atrioventricular cushion formation in Tmem100 null embryos. Dev Dyn. 2015;244:31-42 pubmed publisher
    ..The Tmem100 deficiency causes EndMT defect during AVC cushion formation possibly via disturbance of multiple calcium-related signaling events. ..