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Search Results to Christoph Licht

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One or more keywords matched the following properties of Licht, Christoph

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keywords complement-mediated diseases
overview I am a Professor of Paediatrics at the University of Toronto and Staff Physician in the Division of Nephrology, The Hospital for Sick Children (SickKids). In addition, I am Senior Associate Scientist in the Cell Biology Program of the SickKids Research Institute. I am also cross appointed as Associate Member to the Institute of Medical Science (IMS) and Laboratory Medicine and Pathobiology (LMP) of the University of Toronto. My research focuses on complement-mediated renal diseases such as atypical hemolytic-uremic syndrome (aHUS) and membranoproliferative glomerulonephritis (MPGN) / C3 glomerulopathy (C3G). My research has translational character and includes both clinical and basic research: I have established an international registry with biorepository (www.kidcom.ca). In addition, I have also established a basic research program focusing on the pathogenesis of complement-mediated thrombotic microangiopathy (TMA). In particular, I am investigating the consequences of complement activation on endothelial cells, platelets and neutrophils, and the interaction of these cells in TMA pathogenesis. The results of my research can change our understanding of the pathophysiology of aHUS and may ultimately allow for the development of new treatment strategies not only for aHUS but also other complement-mediated diseases. I am the chair of HUS International (HUSi), and chair of the International aHUS Registry. I serve on the editorial board of Kidney International, Nephrology Dialysis and Transplantation and Pediatric Nephrology, and have published numerous peer-reviewed articles and book chapters related to my research focus. Innovations Advancing diagnosis and treatment of aHUS and related conditions We reported the successful use of eculizumab in aHUS, DEAP HUS, refractory MPGN, antibody-medicated kidney transplant rejection, and post HSCT TMA in children. Modelling vascular endothelium and TMA and aHUS pathology We established blood outgrowth endothelial cells (BOECs) from normal donors and aHUS patients as an approach to study vascular endothelium, in particular the response to complement activation. By using the BioFlux microfluidic system we established a model system to study interactions of endothelium, platelets, neutrophils and complement. A major contribution relates to the investigating of complement and von Willebrand Factor (VWF) identifying VWF as new complement regulator on ECs. Investigating platelet-complement interactions Platelets interact with the AP regulator Factor H (CFH), which is also synthesized by megakaryocytes and can be taken up by platelets in vivo and in vitro. We demonstrated that platelet CFH is functional, bioavailabel and released from stimulated platelets. Investigating neutrophil-endothelium-complement interactions We recently observed that several complement proteins are present in neutrophils and that CRP/properdin mediates complement deposition on NETs and that NETs trap bacteria while complement kills them. We also studied the interaction of neutrophils with complement-challenged endothelial cells, neutrophils and platelets, observing that complement dysregulation stimulates neutrophil adhesion to endothelial cells with subsequent platelet aggregation. Establishing a registry and biorepository for patients with complement-mediated diseases We took a leadership role in establishing an international registry with biorepository that maintains for patients with complement-mediated diseases such as aHUS and C3G (www.kidcom.ca).

One or more keywords matched the following items that are connected to Licht, Christoph

Item TypeName
Concept Complement Hemolytic Activity Assay
Concept Complement C3b Inactivator Proteins
Concept Complement Inactivating Agents
Concept Complement Membrane Attack Complex
Concept Complement C3c
Concept Complement C3b
Concept Complement Factor I
Concept Complement C3 Nephritic Factor
Concept Complement Activation
Concept Complement Pathway, Alternative
Concept Complement Factor H
Concept Complement C3
Concept Complement System Proteins
Concept Complement C5
Concept Complement C3d
Academic Article Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15.
Academic Article Factor H and atypical hemolytic uremic syndrome: mutations in the C-terminus cause structural changes and defective recognition functions.
Academic Article The role of defective complement control in hemolytic uremic syndrome.
Academic Article MPGN II--genetically determined by defective complement regulation?
Academic Article Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome.
Academic Article New approaches to the treatment of dense deposit disease.
Academic Article Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency.
Academic Article C3 deposition glomerulopathy due to a functional factor H defect.
Academic Article Hereditary and acquired complement dysregulation in membranoproliferative glomerulonephritis.
Academic Article Autoimmune forms of thrombotic microangiopathy and membranoproliferative glomerulonephritis: Indications for a disease spectrum and common pathogenic principles.
Academic Article Platelet-associated complement factor H in healthy persons and patients with atypical HUS.
Academic Article Severe atypical HUS caused by CFH S1191L--case presentation and review of treatment options.
Academic Article DEAP-HUS: deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome.
Academic Article aHUS caused by complement dysregulation: new therapies on the horizon.
Academic Article Pandemic H1N1 influenza A infection and (atypical) HUS--more than just another trigger?
Academic Article Genetics of proteinuria: an overview of gene mutations associated with nonsyndromic proteinuric glomerulopathies.
Academic Article Eculizumab and refractory membranoproliferative glomerulonephritis.
Academic Article Antibody mediated rejection associated with complement factor h-related protein 3/1 deficiency successfully treated with eculizumab.
Academic Article An international consensus approach to the management of atypical hemolytic uremic syndrome in children.
Academic Article Abnormalities in the alternative pathway of complement in children with hematopoietic stem cell transplant-associated thrombotic microangiopathy.
Academic Article Complement activation associated with ADAMTS13 deficiency in human and murine thrombotic microangiopathy.
Academic Article Identification of a rare coding variant in complement 3 associated with age-related macular degeneration.
Academic Article Eculizumab in atypical hemolytic-uremic syndrome.
Academic Article Successful treatment of DEAP-HUS with eculizumab.
Academic Article Eculizumab therapy in children with severe hematopoietic stem cell transplantation-associated thrombotic microangiopathy.
Academic Article Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches.
Academic Article An atypical case of acute kidney injury: Hemolytic uremic syndrome (HUS).
Academic Article CFH gene mutation in a case of Shiga toxin-associated hemolytic uremic syndrome (STEC-HUS).
Academic Article Eculizumab is a safe and effective treatment in pediatric patients with atypical hemolytic uremic syndrome.
Academic Article C3 Glomerulopathy and post-infectious glomerulonephritis define a disease spectrum.
Academic Article C3 Glomerulopathy.
Academic Article NETosing Neutrophils Activate Complement Both on Their Own NETs and Bacteria via Alternative and Non-alternative Pathways.
Academic Article The alternative pathway of complement and the thrombotic microangiopathies.
Academic Article Von Willebrand factor regulates complement on endothelial cells.
Academic Article Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement.
Academic Article The role of von Willebrand factor in thrombotic microangiopathy.
Academic Article Complement Activation Induces Neutrophil Adhesion and Neutrophil-Platelet Aggregate Formation on Vascular Endothelial Cells.
Academic Article Relative antibacterial functions of complement and NETs: NETs trap and complement effectively kills bacteria.
Academic Article Clinical and genetic predictors of atypical hemolytic uremic syndrome phenotype and outcome.
Academic Article Outcomes in patients with atypical hemolytic uremic syndrome treated with eculizumab in a long-term observational study.
Academic Article Vascular endothelial cells evade complement-mediated membrane injury via Weibel-Palade body mobilization.
Academic Article Podocytes Produce and Secrete Functional Complement C3 and Complement Factor H.
Academic Article Plasma C3d levels as a diagnostic marker for complete complement factor I deficiency.

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  • Complement C5