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  • Ali BR, Ben-Rebeh I, John A, Akawi NA, Milhem RM, Al-Shehhi NA, Al-Ameri MM, Al-Shamisi SA, Al-Gazali L. Endoplasmic reticulum quality control is involved in the mechanism of endoglin-mediated hereditary haemorrhagic telangiectasia. PLoS One. 2011;6(10):e26206. doi:10.1371/journal.pone.0026206.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Allinson KR, Carvalho RL, van den Brink S, Mummery CL, Arthur HM. Generation of a floxed allele of the mouse Endoglin gene. Genesis. 2007;45:391–5. doi:10.1002/dvg.20284.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Bellón T, Corbí A, Lastres P, Calés C, Cebrián M, Vera S, Cheifetz S, Massague J, Letarte M, Bernabéu C. Identification and expression of two forms of the human transforming growth factor-β-binding protein endoglin with distinct cytoplasmic regions. Eur J Immunol. 1993;23:2340–5. doi:10.1002/eji.1830230943.

    Article  PubMed  Google Scholar 

  • Blanco FJ, Santibanez JF, Guerrero-Esteo M, Langa C, Vary CP, Bernabeu C. Interaction and functional interplay between endoglin and ALK-1, two components of the endothelial transforming growth factor-β receptor complex. J Cell Physiol. 2005;204:574–84. doi:10.1002/jcp.20311.

    Article  CAS  PubMed  Google Scholar 

  • Blanco FJ, Bernabeu C. Alternative splicing factor or splicing factor-2 plays a key role in intron retention of the endoglin gene during endothelial senescence. Aging Cell. 2011;10:896–907. doi:10.1111/j.1474-9726.2011.00727.x.

    Article  CAS  PubMed  Google Scholar 

  • Botella LM, Albiñana V, Ojeda-Fernandez L, Recio-Poveda L, Bernabéu C. Research on potential biomarkers in hereditary hemorrhagic telangiectasia. Front Genet. 2015;6:115. doi:10.3389/fgene.2015.00115.

    Article  PubMed  PubMed Central  Google Scholar 

  • Fernández-Ruiz E, St-Jacques S, Bellón T, Letarte M, Bernabéu C. Assignment of the human endoglin gene (END) to 9q34→qter. Cytogenet Cell Genet. 1993;64:204–7. doi:10.1159/000133576.

    Article  PubMed  Google Scholar 

  • Gougos A, Letarte M. Identification of a human endothelial cell antigen with monoclonal antibody 44G4 produced against a pre-B leukemic cell line. J Immunol. 1988;141:1925–33.

    CAS  PubMed  Google Scholar 

  • Guerrero-Esteo M, Sanchez-Elsner T, Letamendia A, Bernabeu C. Extracellular and cytoplasmic domains of endoglin interact with the transforming growth factor-β receptors I and II. J Biol Chem. 2002;277:29197–209. doi:10.1074/jbc.M111991200.

    Article  CAS  PubMed  Google Scholar 

  • Hawinkels LJ, Kuiper P, Wiercinska E, Verspaget HW, Liu Z, Pardali E, Sier CF, ten Dijke P. Matrix metalloproteinase-14 (MT1-MMP)-mediated endoglin shedding inhibits tumor angiogenesis. Cancer Res. 2010;70:4141–50. doi:10.1158/0008-5472.CAN-09-4466.

    Article  CAS  PubMed  Google Scholar 

  • Jovine L, Darie CC, Litscher ES, Wassarman PM. Zona pellucida domain proteins. Annu Rev Biochem. 2005;74:83–114. doi:10.1146/annurev.biochem.74.082803.133039.

    Article  CAS  PubMed  Google Scholar 

  • Koleva RI, Conley BA, Romero D, Riley KS, Marto JA, Lux A, Vary CP. Endoglin structure and function: determinants of endoglin phosphorylation by transforming growth factor-β receptors. J Biol Chem. 2006;281:25110–23. doi:10.1074/jbc.M601288200.

    Article  CAS  PubMed  Google Scholar 

  • Lastres P, Martín-Perez J, Langa C, Bernabéu C. Phosphorylation of the human-transforming-growth-factor-β-binding protein endoglin. Biochem J. 1994;301:765–8. doi:10.1042/bj3010765.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Lee NY, Blobe GC. The interaction of endoglin with β-arrestin2 regulates transforming growth factor-beta-mediated ERK activation and migration in endothelial cells. J Biol Chem. 2007;282:21507–17. doi:10.1074/jbc.M700176200.

    Article  CAS  PubMed  Google Scholar 

  • Lee NY, Ray B, How T, Blobe GC. Endoglin promotes transforming growth factor beta-mediated Smad 1/5/8 signaling and inhibits endothelial cell migration through its association with GIPC. J Biol Chem. 2008;283:32527–33. doi:10.1074/jbc.M803059200.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Letamendía A, Lastres P, Botella LM, Raab U, Langa C, Velasco B, Attisano L, Bernabeu C. Role of endoglin in cellular responses to transforming growth factor-β. A comparative study with betaglycan. J Biol Chem. 1998;273:33011–9. doi:10.1074/jbc.273.49.33011.

    Article  PubMed  Google Scholar 

  • Llorca O, Trujillo A, Blanco FJ, Bernabeu C. Structural model of human endoglin, a transmembrane receptor responsible for hereditary hemorrhagic telangiectasia. J Mol Biol. 2007;365:694–705. doi:10.1016/j.jmb.2006.10.015.

    Article  CAS  PubMed  Google Scholar 

  • Lux A, Müller R, Tulk M, Olivieri C, Zarrabeita R, Salonikios T, Wirnitzer B. HHT diagnosis by Mid-infrared spectroscopy and artificial neural network analysis. Orphanet J Rare Dis. 2013;8:94. doi:10.1186/1750-1172-8-94.

    Article  PubMed  PubMed Central  Google Scholar 

  • McAllister KA, Grogg KM, Johnson DW, Gallione CJ, Baldwin MA, Jackson CE, Helmbold EA, Markel DS, McKinnon WC, Murrell J. Endoglin, a TGF-β binding protein of endothelial cells, is the gene for hereditary haemorrhagic telangiectasia type 1. Nat Genet. 1994;8:345–51. doi:10.1038/ng1294-345.

    Article  CAS  PubMed  Google Scholar 

  • Meurer SK, Tihaa L, Lahme B, Gressner AM, Weiskirchen R. Identification of endoglin in rat hepatic stellate cells: new insights into transforming growth factor β receptor signaling. J Biol Chem. 2005;280:3078–87. doi:10.1074/jbc.M405411200.

    Article  CAS  PubMed  Google Scholar 

  • Meurer SK, Tihaa L, Borkham-Kamphorst E, Weiskirchen R. Expression and functional analysis of endoglin in isolated liver cells and its involvement in fibrogenic Smad signalling. Cell Signal. 2011;23:683–99. doi:10.1016/j.cellsig.2010.12.002.

    Article  CAS  PubMed  Google Scholar 

  • Meurer SK, Alsamman M, Sahin H, Wasmuth HE, Kisseleva T, Brenner DA, Trautwein C, Weiskirchen R, Scholten D. Overexpression of endoglin modulates TGF-β1-signalling pathways in a novel immortalized mouse hepatic stellate cell line. PLoS One. 2013;8(2):e56116. doi:10.1371/journal.pone.0056116.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Meurer SK, Alsamman M, Scholten D, Weiskirchen R. Endoglin in liver fibrogenesis: bridging basic science and clinical practice. World J Biol Chem. 2014;5:180–203. doi:10.4331/wjbc.v5.i2.180.

    PubMed  PubMed Central  Google Scholar 

  • Ojeda-Fernández L, Recio-Poveda L, Aristorena M, Lastres P, Blanco FJ, Sanz-Rodríguez F, Gallardo-Vara E, de Las Casas-Engel M, Corbí Á, Arthur HM, Bernabeu C, Botella LM. Mice lacking endoglin in macrophages show an impaired immune response. PLoS Genet. 2016;12(3):e1005935. doi:10.1371/journal.pgen.1005935.

    Article  PubMed  PubMed Central  Google Scholar 

  • Olitsky SE. Hereditary hemorrhagic telangiectasia: diagnosis and management. Am Fam Physician. 2010;82:785–90.

    PubMed  Google Scholar 

  • Pan CC, Kumar S, Shah N, Hoyt DG, Hawinkels LJ, Mythreye K, Lee NY. Src-mediated post-translational regulation of endoglin stability and function is critical for angiogenesis. J Biol Chem. 2014;289:25486–96. doi:10.1074/jbc.M114.578609.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Pérez-Gómez E, Eleno N, López-Novoa JM, Ramirez JR, Velasco B, Letarte M, Bernabéu C, Quintanilla M. Characterization of murine S-endoglin isoform and its effects on tumor development. Oncogene. 2005;24:4450–61. doi:10.1038/sj.onc.1208644.

    Article  PubMed  Google Scholar 

  • Pomeraniec L, Hector-Greene M, Ehrlich M, Blobe GC, Henis YI. Regulation of TGF-β receptor hetero-oligomerization and signaling by endoglin. Mol Biol Cell. 2015;26:3117–27. doi:10.1091/mbc.E15-02-0069.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  • Qureshi ST, Gros P, Letarte M, Malo D. The murine endoglin gene (Eng) maps to chromosome 2. Genomics. 1995;26:165–6. doi:10.1016/0888-7543(95)80099-8.

    Article  CAS  PubMed  Google Scholar 

  • Ray BN, Lee NY, How T, Blobe GC. ALK5 phosphorylation of the endoglin cytoplasmic domain regulates Smad1/5/8 signaling and endothelial cell migration. Carcinogenesis. 2010;31:435–41. doi:10.1093/carcin/bgp327.

    Article  CAS  PubMed  Google Scholar 

  • Sanz-Rodriguez F, Guerrero-Esteo M, Botella LM, Banville D, Vary CP, Bernabéu C. Endoglin regulates cytoskeletal organization through binding to ZRP-1, a member of the Lim family of proteins. J Biol Chem. 2004;279:32858–68. doi:10.1074/jbc.M400843200.

    Article  CAS  PubMed  Google Scholar 

  • Scherner O, Meurer SK, Tihaa L, Gressner AM, Weiskirchen R. Endoglin differentially modulates antagonistic transforming growth factor-β1 and BMP-7 signaling. J Biol Chem. 2007;282:13934–43. doi:10.1074/jbc.M611062200.

    Article  CAS  PubMed  Google Scholar 

  • Segatelli V, de Oliveira EC, Boin IF, Ataide EC, Escanhoela CA. Evaluation and comparison of microvessel density using the markers CD34 and CD105 in regenerative nodules, dysplastic nodules and hepatocellular carcinoma. Hepatol Int. 2014;8:260–5. doi:10.1007/s12072-014-9525-9.

    PubMed  Google Scholar 

  • Shovlin CL, Guttmacher AE, Buscarini E, Faughnan ME, Hyland RH, Westermann CJ, Kjeldsen AD, Plauchu H. Diagnostic criteria for hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber syndrome). Am J Med Genet. 2000;91:66–7. doi:10.1002/(SICI)1096-8628(20000306)91:1<66::AID-AJMG12>3.0.CO;2-P.

    Article  CAS  PubMed  Google Scholar 

  • Tual-Chalot S, Oh SP, Arthur HM. Mouse models of hereditary hemorrhagic telangiectasia: recent advances and future challenges. Front Genet. 2015;6:25. doi:10.3389/fgene.2015.00025.

    Article  PubMed  PubMed Central  Google Scholar 

  • Xu G, Barrios-Rodiles M, Jerkic M, Turinsky AL, Nadon R, Vera S, Voulgaraki D, Wrana JL, Toporsian M, Letarte M. Novel protein interactions with endoglin and activin receptor-like kinase 1: potential role in vascular networks. Mol Cell Proteomics. 2014;13:489–502. doi:10.1074/mcp.M113.033464.

    Article  CAS  PubMed  Google Scholar 

  • Yao Y, Pan Y, Chen J, Sun X, Qiu Y, Ding Y. Endoglin (CD105) expression in angiogenesis of primary hepatocellular carcinomas: analysis using tissue microarrays and comparisons with CD34 and VEGF. Ann Clin Lab Sci. 2007;37:39–48.

    CAS  PubMed  Google Scholar 

  • Yagmur E, Rizk M, Stanzel S, Hellerbrand C, Lammert F, Trautwein C, Wasmuth HE, Gressner AM. Elevation of endoglin (CD105) concentrations in serum of patients with liver cirrhosis and carcinoma. Eur J Gastroenterol Hepatol. 2007;19:755–61. doi:10.1097/MEG.0b013e3282202bea.

    Article  CAS  PubMed  Google Scholar 

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Acknowledgments

RW is supported by grants from the German Research Foundation (SFB/TRR57, P13/Q3) and a grant from the Interdisciplinary Centre for Clinical Research within the Faculty of Medicine at the RWTH Aachen University (IZKF Aachen, Project E7-6). The authors are grateful to Sabine Weiskirchen for preparing line drawings, Dr. J. MacDonald (Department of Radiology, Hereditary Hemorrhagic Telangiectasia Center, University of Utah, Salt Lake City, UT, USA) and Dr. U. Geisthoff (Department of Otorhinolaryngology, Essen University Hospital, Essen, Germany) for providing photographs. In addition, the authors would like to thank the colleagues Prof. Frank Gaillard, Dr. Andrew Dixon, and Dr. Nasir Siddiqui that deposited radiology images in the Radiopaedia.org resource and allowed us to use them in our work.

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Meurer, S.K., Weiskirchen, R. (2016). ENG. In: Choi, S. (eds) Encyclopedia of Signaling Molecules. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6438-9_101593-1

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