A5021541588- Vascular Anomalies and Treatments
- Tracheal and airway disorders
- Cancer, Hypoxia, and Metabolism
- Sharing Economy and Platforms
- Pulmonary Hypertension Research and Treatments
- Vascular Malformations and Hemangiomas
- Kruppel-like factors research
- Cardiac tumors and thrombi
- TGF-β signaling in diseases
- Insect Resistance and Genetics
- Insect and Arachnid Ecology and Behavior
- Ocular Oncology and Treatments
- Angiogenesis and VEGF in Cancer
- Cell Adhesion Molecules Research
- Cancer-related gene regulation
- Otitis Media and Relapsing Polychondritis
- Esophageal and GI Pathology
- Neurobiology and Insect Physiology Research
- Protist diversity and phylogeny
- Invertebrate Immune Response Mechanisms
- Genetic and Kidney Cyst Diseases
- Fibroblast Growth Factor Research
- Vascular Malformations Diagnosis and Treatment
- Hemostasis and retained surgical items
- Congenital heart defects research
Centro de Investigaciones Biológicas Margarita Salas
2015-2024
Centre for Biomedical Network Research on Rare Diseases
2014-2024
Centro de Investigación Biomédica en Red
2015-2024
Instituto de Salud Carlos III
2008-2024
Consejo Superior de Investigaciones Científicas
2013-2024
Instituto de Investigación de Enfermedades Raras
2008-2024
The University of Tokyo
2012
National Research Council
2007
Universidad Autónoma de Madrid
2006
Fernandez Hospital
2006
Signaling by transforming growth factor (TGF)-β family members is mediated Smad proteins that regulate gene transcription through functional cooperativity and association with other DNA-binding proteins. The hypoxia-inducible (HIF)-1 a transcriptional complex plays key role in oxygen-regulated expression. We demonstrate hypoxia TGF-β cooperate the induction of promoter activity vascular endothelial (VEGF), which major stimulus promotion angiogenesis. This cooperation has been mapped on human...
Endoglin (CD105) is the target gene for hereditary hemorrhagic telangiectasia type I (HHT1), a dominantly inherited vascular disorder. It shares with betaglycan limited amino acid sequence homology and being components of membrane transforming growth factor-β (TGF-β) receptor complex. Using rat myoblasts as model system, we found that overexpression endoglin led to decreased TGF-β response cellular inhibition plasminogen activator inhibitor-1 synthesis, whereas resulted in an enhanced...
Objective— ALK1 (activin-receptor like kinase 1) is an endothelial cell-restricted receptor with high affinity for BMP (bone morphogenetic protein) 9 TGF-β (transforming growth factor-β) family member. Loss-of-function mutations in cause a subtype of hereditary hemorrhagic telangiectasia—a rare disease characterized by vasculature malformations. Therapeutic strategies are aimed at reducing potential complications because vascular malformations, but currently, there no curative treatment...
Hereditary hemorrhagic telangiectasia (HHT) is a multisystemic inherited vascular dysplasia that leads to nosebleeds and visceral arteriovenous malformations (AVMs). Anti-angiogenic drugs thalidomide bevacizumab have been increasingly used off-label with variable results. The HHT working group within the ERN for Rare Multisystemic Vascular Diseases (VASCERN), developed questionnaire-based retrospective capture of adverse events (AEs) classified using Common Terminology Criteria Adverse...
The circulatory system is walled off by different cell types, including vascular mural cells and podocytes. interaction interplay between endothelial (ECs) cells, such as smooth muscle or pericytes, play a pivotal role in biology. Endoglin an RGD-containing counter-receptor for β1 integrins highly expressed ECs during angiogenesis. We find that the adhesion enhanced integrin activators inhibited upon suppression of membrane endoglin β1-integrin, well addition soluble (SolEng), anti-integrin...
Upon inflammation, monocyte-derived macrophages (MΦ) infiltrate blood vessels to regulate several processes involved in vascular pathophysiology. However, little is known about the mediators involved. Macrophage polarization crucial for a fast and efficient initial response (GM-MΦ) good resolution (M-MΦ) of inflammatory process. The functional activity polarized MΦ exerted mainly through their secretome, which can target other cell types, including endothelial cells. Endoglin (CD105) surface...
After endothelial injury, the transcription factor Krüppel-like 6 (KLF6) translocates into cell nucleus to regulate a variety of target genes involved in angiogenesis, vascular repair and remodeling, including components membrane transforming growth beta (TGF-β) receptor complex such as endoglin activin receptor-like kinase 1. The metalloproteinase 14 (MMP14 or MT1-MMP) targets release soluble is inflammation tubulogenesis. However, little known about regulation MMP14 expression during...
Hereditary haemorrhagic telangiectasia (HHT) is originated by mutations in endoglin (HHT1) and ALK1 (HHT2) genes. The purpose of this work was to isolate characterize circulating endothelial cells from HHT patients.Pure primary cultures blood outgrowth (BOECs) were obtained 50 ml peripheral selection on collagen plates with medium.The amount HHT1-BOECs half the controls, but HHT2-BOECs are also endoglin-deficient. Since TGF-beta/ALK1 pathway activates promoter activity, these results suggest...
Summary Hereditary haemorrhagic telangiectasia (HHT), or Rendu-Osler-Weber syndrome, is an autosomal dominant vascular disease. The clinical manifestations are epistaxis, mucocutaneous and gastrointestinal telangiectases, arteriovenous malformations. There two predominant types of HHT caused by mutations in Endoglin (ENG) activin receptor-like kinase 1 (ALK1) (ACVRL1) genes, HHT1 HHT2, respectively. No cure for has been found there a current need to find new effective drug treatments the...
Activin receptor-like kinase-1 (ALK1) is an endothelial transforming growth factor β receptor involved in angiogenesis. ALK1 expression high the embryo vasculature, becoming less detectable quiescent endothelium of adult stages. However, becomes rapidly increased after angiogenic stimuli such as vascular injury.To characterize molecular mechanisms underlying regulation on injury.Alk1 strongly upregulated (EC) and smooth muscle cells mouse femoral arteries wire-induced denudation. In vitro...
Endoglin is an auxiliary receptor for members of the TGF-β superfamily and plays important role in homeostasis vessel wall. Mutations endoglin gene (ENG) or closely related type I ACVRL1/ALK1 are responsible a rare dominant vascular dysplasia, Hereditary Hemorrhagic Telangiectasia (HHT), Rendu-Osler-Weber syndrome. also expressed human macrophages, but its macrophage function remains unknown. In this work, we show that expression triggered during monocyte-macrophage differentiation process,...
There are very few studies about general quality of life parameters, standards for the description health status and comparison with population data on patients Hereditary hemorrhagic telangiectasia (HHT), a rare disease in which epistaxis is cardinal symptom. To assess Spanish HHT compare it population. Between January 1st 2005 December 31st 2013, 187 adult diagnosed who were admitted to Unit Hospital Sierrallana, completed their first visit, EuroQol 5D-3L (five dimensions three levels)...
Hereditary haemorrhagic telangiectasia (HHT) is a rare vascular multisystemic disease that leads to epistaxis, anaemia due blood loss, and arteriovenous malformations (AVMs) in organs such as the lungs, liver brain. HHT prevalence estimated at 1/6000, i.e. around 85,000 European citizens, served by Reference Network for Rare Multisystemic Vascular Diseases (VASCERN). treatments depend on clinical manifestations, span multiple different medical, surgical interventional disciplines. Separate...
The CD11c/CD18 integrin binds lipopolysaccharide, fibrinogen, and heparin, mediates leukocyte adhesion, spreading, migration. is primarily found on myeloid cells its expression regulated during differentiation by transcriptional mechanisms acting the CD11c gene promoter. We now describe that CCAAT/enhancer-binding proteins (C/EBP) contribute to basal, tissue-specific developmentally activity of A C/EBP-binding site within promoter (CEBP-80) bound CEBPα in undifferentiated U937 C/EBPα-...