Endothelial cells: source, barrier, and target of defensive mediators

Regulators of complement activity mediate inhibitory mechanisms through a common C3b‐binding mode

(2016) Federico Forneris et al.   EMBO JOURNAL

Complement genetics and susceptibility to inflammatory disease. Lessons from genotype–phenotype correlations

(2016) Santiago Rodríguez de Córdoba   IMMUNOBIOLOGY

Mutations in Complement Factor H Impair Alternative Pathway Regulation on Mouse Glomerular Endothelial Cellsin Vitro

(2016) Markus A. Loeven et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Von Willebrand factor regulates complement on endothelial cells

(2016) Damien G. Noone et al.   KIDNEY INTERNATIONAL

Heterogeneity but individual constancy of epitopes, isotypes and avidity of factor H autoantibodies in atypical hemolytic uremic syndrome

(2016) Pilar Nozal et al.   MOLECULAR IMMUNOLOGY

Complement gene variants determine the risk of immunoglobulin-associated MPGN and C3 glomerulopathy and predict long-term renal outcome

(2016) Paraskevas Iatropoulos et al.   MOLECULAR IMMUNOLOGY

T helper 1 immunity requires complement-driven NLRP3 inflammasome activity in CD4+ T cells

(2016) G. Arbore et al.   SCIENCE

Defining the genetics of thrombotic microangiopathies

(2016) Paula Vieira-Martins et al.   TRANSFUSION AND APHERESIS SCIENCE

Tissue factor as a link between inflammation and coagulation

(2016) Marco Witkowski et al.   TRENDS IN CARDIOVASCULAR MEDICINE

Heme: Modulator of Plasma Systems in Hemolytic Diseases

(2016) Lubka T. Roumenina et al.   TRENDS IN MOLECULAR MEDICINE

Inhibition of complement component C5 prevents clotting in an ex vivo model of xenogeneic activation of coagulation

(2016) Dennis Rataj et al.   XENOTRANSPLANTATION

C1q acts in the tumour microenvironment as a cancer-promoting factor independently of complement activation

(2016) Roberta Bulla et al.   Nature Communications

A role of stochastic phenotype switching in generating mosaic endothelial cell heterogeneity

(2016) Lei Yuan et al.   Nature Communications

Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndrome

(2015) E. C. Schramm et al.   BLOOD

Eculizumab reduces complement activation, inflammation, endothelial damage, thrombosis, and renal injury markers in aHUS

(2015) R. Cofiell et al.   BLOOD

von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends

(2015) P. J. Lenting et al.   BLOOD

Platelet secretion: From haemostasis to wound healing and beyond

(2015) Ewelina M. Golebiewska et al.   BLOOD REVIEWS

Fibrinolysis and the control of blood coagulation

(2015) John C. Chapin et al.   BLOOD REVIEWS

C5a alters blood-brain barrier integrity in a humanin vitromodel of systemic lupus erythematosus

(2015) Supriya D. Mahajan et al.   IMMUNOLOGY

Prevalence and Gene Characteristics of Antibodies with Cofactor-induced HIV-1 Specificity

(2015) Maxime Lecerf et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

TNF Regulates Essential Alternative Complement Pathway Components and Impairs Activation of Protein C in Human Glomerular Endothelial Cells

(2015) Sarah E. Sartain et al.   JOURNAL OF IMMUNOLOGY

Is the Complement Activation Product C3a a Proinflammatory Molecule? Re-evaluating the Evidence and the Myth

(2015) Liam G. Coulthard et al.   JOURNAL OF IMMUNOLOGY

Partial Complement Factor H Deficiency Associates with C3 Glomerulopathy and Thrombotic Microangiopathy

(2015) K. A. Vernon et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H

(2015) S. Chauvet et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Uremic Toxicity of Advanced Glycation End Products in CKD

(2015) A. E. M. Stinghen et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

The role of heparan sulfate as determining pathogenic factor in complement factor H-associated diseases

(2015) Markus A. Loeven et al.   MOLECULAR IMMUNOLOGY

Complement factor H, FHR-3 and FHR-1 variants associate in an extended haplotype conferring increased risk of atypical hemolytic uremic syndrome

(2015) Maria E. Bernabéu-Herrero et al.   MOLECULAR IMMUNOLOGY

The molecular and structural bases for the association of complement C3 mutations with atypical hemolytic uremic syndrome

(2015) Rubén Martínez-Barricarte et al.   MOLECULAR IMMUNOLOGY

Atypical aHUS: State of the art

(2015) Carla M. Nester et al.   MOLECULAR IMMUNOLOGY

Complement, a target for therapy in inflammatory and degenerative diseases

(2015) B. Paul Morgan et al.   NATURE REVIEWS DRUG DISCOVERY

The efficacy of recombinant human soluble thrombomodulin for the treatment of shiga toxin-associated hemolytic uremic syndrome model mice

(2015) K. Suyama et al.   NEPHROLOGY DIALYSIS TRANSPLANTATION

Atypical haemolytic uraemic syndrome in a Japanese patient with DGKE genetic mutations

(2015) Yumiko Uchida et al.   THROMBOSIS AND HAEMOSTASIS

Complementing the Sugar Code: Role of GAGs and Sialic Acid in Complement Regulation

(2015) Alex Langford-Smith et al.   Frontiers in Immunology

Complement System Part II: Role in Immunity

(2015) Nicolas S. Merle et al.   Frontiers in Immunology

Complement System Part I – Molecular Mechanisms of Activation and Regulation

(2015) Nicolas S. Merle et al.   Frontiers in Immunology

Regulatory Components of the Alternative Complement Pathway in Endothelial Cell Cytoplasm, Factor H and Factor I, Are Not Packaged in Weibel-Palade Bodies

(2015) Nancy A. Turner et al.   PLoS One

von Willebrand factor is a cofactor in complement regulation

(2014) S. Feng et al.   BLOOD

Loss of DGK  induces endothelial cell activation and death independently of complement activation

(2014) S. Bruneau et al.   BLOOD

Dynamics of complement activation in aHUS and how to monitor eculizumab therapy

(2014) M. Noris et al.   BLOOD

Phenotypic Expansion of DGKE-Associated Diseases

(2014) R. Westland et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Complement Factor B Mutations in Atypical Hemolytic Uremic Syndrome--Disease-Relevant or Benign?

(2014) M. C. Marinozzi et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Structural basis for sialic acid–mediated self-recognition by complement factor H

(2014) Bärbel S Blaum et al.   Nature Chemical Biology

C1q as a unique player in angiogenesis with therapeutic implication in wound healing

(2014) F. Bossi et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Modulation of age-related insulin sensitivity by VEGF-dependent vascular plasticity in adipose tissues

(2014) J. Honek et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Thrombomodulin and the vascular endothelium: insights into functional, regulatory, and therapeutic aspects

(2013) Fiona A. Martin et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Complement Genes Strongly Predict Recurrence and Graft Outcome in Adult Renal Transplant Recipients with Atypical Hemolytic and Uremic Syndrome

(2013) M. Le Quintrec et al.   AMERICAN JOURNAL OF TRANSPLANTATION

Eculizumab Improves Posttransplant Thrombotic Microangiopathy Due to Antiphospholipid Syndrome Recurrence but Fails to Prevent Chronic Vascular Changes

(2013) G. Canaud et al.   AMERICAN JOURNAL OF TRANSPLANTATION

Human Complement Factor H Is a Reductase for Large Soluble von Willebrand Factor Multimers—Brief Report

(2013) Leticia Nolasco et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

The interaction between factor H and VWF increases factor H cofactor activity and regulates VWF prothrombotic status

(2013) J. Rayes et al.   BLOOD

Complement activation by heme as a secondary hit for atypical hemolytic uremic syndrome

(2013) M. Frimat et al.   BLOOD

Heme triggers TLR4 signaling leading to endothelial cell activation and vaso-occlusion in murine sickle cell disease

(2013) J. D. Belcher et al.   BLOOD

Genetics and Outcome of Atypical Hemolytic Uremic Syndrome: A Nationwide French Series Comparing Children and Adults

(2013) V. Fremeaux-Bacchi et al.   Clinical Journal of the American Society of Nephrology

Molecular Signatures of Tissue-Specific Microvascular Endothelial Cell Heterogeneity in Organ Maintenance and Regeneration

(2013) Daniel J. Nolan et al.   DEVELOPMENTAL CELL

The endothelial glycocalyx as a potential modifier of the hemolytic uremic syndrome

(2013) Margien G.S. Boels et al.   European Journal of Internal Medicine

Recombinant soluble human thrombomodulin (thrombomodulin alfa) in the treatment of neonatal disseminated intravascular coagulation

(2013) Akira Shirahata et al.   EUROPEAN JOURNAL OF PEDIATRICS

Intracellular Complement Activation Sustains T Cell Homeostasis and Mediates Effector Differentiation

(2013) M. Kathryn Liszewski et al.   IMMUNITY

von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin αIIbβ3

(2013) Caterina Casari et al.   JOURNAL OF CLINICAL INVESTIGATION

An Engineered Construct Combining Complement Regulatory and Surface-Recognition Domains Represents a Minimal-Size Functional Factor H

(2013) M. Hebecker et al.   JOURNAL OF IMMUNOLOGY

Antibody Polyreactivity in Health and Disease: Statu Variabilis

(2013) J. D. Dimitrov et al.   JOURNAL OF IMMUNOLOGY

Comprehensive Genetic Analysis of Complement and Coagulation Genes in Atypical Hemolytic Uremic Syndrome

(2013) F. Bu et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Combined Complement Gene Mutations in Atypical Hemolytic Uremic Syndrome Influence Clinical Phenotype

(2013) E. Bresin et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Recessive mutations in DGKE cause atypical hemolytic-uremic syndrome

(2013) Mathieu Lemaire et al.   NATURE GENETICS

Terminal Complement Inhibitor Eculizumab in Atypical Hemolytic–Uremic Syndrome

(2013) C.M. Legendre et al.   NEW ENGLAND JOURNAL OF MEDICINE

A Novel Strategy for Hemolytic Uremic Syndrome: Successful Treatment With Thrombomodulin  

(2013) T. Honda et al.   PEDIATRICS

New Fundamentals in Hemostasis

(2013) Henri H. Versteeg et al.   PHYSIOLOGICAL REVIEWS

Assembly and Activation of Alternative Complement Components on Endothelial Cell-Anchored Ultra-Large Von Willebrand Factor Links Complement and Hemostasis-Thrombosis

(2013) Nancy A. Turner et al.   PLoS One

Comprehensive analysis of glomerular mRNA expression of pro- and antithrombotic genes in atypical haemolytic-uremic syndrome (aHUS)

(2013) Friedrich Modde et al.   VIRCHOWS ARCHIV

Brief Report: Induction of sustained remission in recurrent catastrophic antiphospholipid syndrome via inhibition of terminal complement with eculizumab

(2012) Iuliana Shapira et al.   ARTHRITIS AND RHEUMATISM

Thrombin generates previously unidentified C5 products that support the terminal complement activation pathway

(2012) M. J. Krisinger et al.   BLOOD

A prevalent C3 mutation in aHUS patients causes a direct C3 convertase gain of function

(2012) L. T. Roumenina et al.   BLOOD

Improving on nature: redesigning ADAMTS13

(2012) J. A. K. Hovinga et al.   BLOOD

Dynamical Systems Approach to Endothelial Heterogeneity

(2012) Erzsébet Ravasz Regan et al.   CIRCULATION RESEARCH

Familial Atypical Hemolytic Uremic Syndrome: A Review of Its Genetic and Clinical Aspects

(2012) Fengxiao Bu et al.   Clinical & Developmental Immunology

Causes of Alternative Pathway Dysregulation in Dense Deposit Disease

(2012) Y. Zhang et al.   Clinical Journal of the American Society of Nephrology

In Vivo Imaging of Cerebral Microvascular Plasticity from Birth to Death

(2012) Roa Harb et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Overall Neutralization of Complement Factor H by Autoantibodies in the Acute Phase of the Autoimmune Form of Atypical Hemolytic Uremic Syndrome

(2012) C. Blanc et al.   JOURNAL OF IMMUNOLOGY

Complement activation in thrombotic thrombocytopenic purpura

(2012) M. RÉTI et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies

(2012) Aude Servais et al.   KIDNEY INTERNATIONAL

Sensitive and specific assays for C3 nephritic factors clarify mechanisms underlying complement dysregulation

(2012) Danielle Paixão-Cavalcante et al.   KIDNEY INTERNATIONAL

STEC-HUS, atypical HUS and TTP are all diseases of complement activation

(2012) Marina Noris et al.   Nature Reviews Nephrology

Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies

(2012) Julien Zuber et al.   Nature Reviews Nephrology

Renal and neurological involvement in typical Shiga toxin-associated HUS

(2012) Howard Trachtman et al.   Nature Reviews Nephrology

Novel C3 mutation p.Lys65Gln in aHUS affects complement factor H binding

(2012) Elena Volokhina et al.   PEDIATRIC NEPHROLOGY

Genetics of hemolytic uremic syndromes

(2012) Michal Malina et al.   PRESSE MEDICALE

Human Leukocyte Antigen-Specific Antibodies and Gamma-Interferon Stimulate Human Microvascular and Glomerular Endothelial Cells to Produce Complement Factor C4

(2012) Rizwan Hamer et al.   TRANSPLANTATION

Age-Related Penetrance of Hereditary Atypical Hemolytic Uremic Syndrome

(2011) Maren Sullivan et al.   ANNALS OF HUMAN GENETICS

Pathogenesis of Antineutrophil Cytoplasmic Autoantibody–Associated Small-Vessel Vasculitis

(2011) J. Charles Jennette et al.   Annual Review of Pathology-Mechanisms of Disease

Regulation and Functions of Diacylglycerol Kinases

(2011) Yulia V. Shulga et al.   CHEMICAL REVIEWS

Substrate specificity of diacylglycerol kinase-epsilon and the phosphatidylinositol cycle

(2011) Yulia V. Shulga et al.   FEBS LETTERS

Heme Interacts with C1q and Inhibits the Classical Complement Pathway

(2011) Lubka T. Roumenina et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Alternative complement pathway assessment in patients with atypical HUS

(2011) Lubka T. Roumenina et al.   JOURNAL OF IMMUNOLOGICAL METHODS

Alternative Pathway Activation of Complement by Shiga Toxin Promotes Exuberant C3a Formation That Triggers Microvascular Thrombosis

(2011) M. Morigi et al.   JOURNAL OF IMMUNOLOGY

Structural basis for engagement by complement factor H of C3b on a self surface

(2011) Hugh P Morgan et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

Dual interaction of factor H with C3d and glycosaminoglycans in host-nonhost discrimination by complement

(2011) T. Kajander et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Endothelium—role in regulation of coagulation and inflammation

(2011) Victor W. M. van Hinsbergh   Seminars in Immunopathology

Repressors NFI and NFY Participate in Organ-Specific Regulation of von Willebrand Factor Promoter Activity in Transgenic Mice

(2010) Marjan Nassiri et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

The Anti-Inflammatory Cytokine Interleukin 19 Is Expressed By and Angiogenic for Human Endothelial Cells

(2010) Surbhi Jain et al.   ARTERIOSCLEROSIS THROMBOSIS AND VASCULAR BIOLOGY

Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B

(2010) J. Rayes et al.   BLOOD

A revised model for the secretion of tPA and cytokines from cultured endothelial cells

(2010) L. Knipe et al.   BLOOD

Complement alternative pathway acts as a positive feedback amplification of neutrophil activation

(2010) L. Camous et al.   BLOOD

Activation of the endothelium by IL-1α and glucocorticoids results in major increase of complement C3 and factor B production and generation of C3a

(2010) M. COULPIER et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Translational Mini-Review Series on Complement Factor H: Renal diseases associated with complement factor H: novel insights from humans and animals

(2010) M. C. Pickering et al.   CLINICAL AND EXPERIMENTAL IMMUNOLOGY

Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype

(2010) M. Noris et al.   Clinical Journal of the American Society of Nephrology

Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome

(2010) Tara K. Maga et al.   HUMAN MUTATION

Pregnancy-Associated Hemolytic Uremic Syndrome Revisited in the Era of Complement Gene Mutations

(2010) F. Fakhouri et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

The Development of Atypical Hemolytic Uremic Syndrome Depends on Complement C5

(2010) E. G. de Jorge et al.   JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY

Tissue factor up-regulation in proinflammatory conditions confers thrombin generation capacity to endothelial colony-forming cells without influencing non-coagulant properties in vitro

(2010) W. CUCCUINI et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

Complement in atherosclerosis: friend or foe?

(2010) W. S. SPEIDL et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

C3 glomerulopathy: a new classification

(2010) Fadi Fakhouri et al.   Nature Reviews Nephrology

Properdin: Emerging Roles of a Pattern-Recognition Molecule

(2009) Claudia Kemper et al.   Annual Review of Immunology

Contributions of extravascular and intravascular cells to fibrin network formation, structure, and stability

(2009) R. A. Campbell et al.   BLOOD

Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome

(2009) L. T. Roumenina et al.   BLOOD

Mutations in complement factor I as found in atypical hemolytic uremic syndrome lead to either altered secretion or altered function of factor I

(2009) Sara C. Nilsson et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

Activation by C5a of endothelial cell caspase 8 and cFLIP

(2009) E. A. Albrecht et al.   INFLAMMATION RESEARCH

ADP Signaling in Vascular Endothelial Cells

(2009) Connie Ng Hess et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Pathologic Continuum of Diabetic Vascular Disease

(2009) Gabriela Orasanu et al.   JOURNAL OF THE AMERICAN COLLEGE OF CARDIOLOGY

Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome

(2009) Frank Bienaime et al.   KIDNEY INTERNATIONAL

Structure of complement fragment C3b–factor H and implications for host protection by complement regulators

(2009) Jin Wu et al.   NATURE IMMUNOLOGY

Weibel–Palade bodies—sentinels of acute stress

(2009) Michael S. Goligorsky et al.   Nature Reviews Nephrology

Thrombomodulin Mutations in Atypical Hemolytic–Uremic Syndrome

(2009) Mieke Delvaeye et al.   NEW ENGLAND JOURNAL OF MEDICINE

Atypical Hemolytic–Uremic Syndrome

(2009) Marina Noris et al.   NEW ENGLAND JOURNAL OF MEDICINE

In vivo differences between endothelial transcriptional profiles of coronary and iliac arteries revealed by microarray analysis

(2008) Ji Zhang et al.   AMERICAN JOURNAL OF PHYSIOLOGY-HEART AND CIRCULATORY PHYSIOLOGY

Mutations in complement C3 predispose to development of atypical hemolytic uremic syndrome

(2008) V. Fremeaux-Bacchi et al.   BLOOD

Heme induces endothelial tissue factor expression: potential role in hemostatic activation in patients with hemolytic anemia

(2008) B. N. Y. SETTY et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

ADAMTS-13 cleaves long von Willebrand factor multimeric strings anchored to endothelial cells in the absence of flow, platelets or conformation-altering chemicals

(2008) N. TURNER et al.   JOURNAL OF THROMBOSIS AND HAEMOSTASIS

Decidual endothelial cells express surface-bound C1q as a molecular bridge between endovascular trophoblast and decidual endothelium

(2008) Roberta Bulla et al.   MOLECULAR IMMUNOLOGY

Endothelial cells are a target of both complement and kinin system

(2007) Fleur Bossi et al.   INTERNATIONAL IMMUNOPHARMACOLOGY