Applying complement therapeutics to rare diseases.

Complement in disease: a defence system turning offensive. Nat Rev Nephrol (2016) 0.97

Structural basis for therapeutic inhibition of complement C5. Nat Struct Mol Biol (2016) 0.83

New milestones ahead in complement-targeted therapy. Semin Immunol (2016) 0.81

From orphan drugs to adopted therapies: Advancing C3-targeted intervention to the clinical stage. Immunobiology (2016) 0.80

Anti-Immune Strategies of Pathogenic Fungi. Front Cell Infect Microbiol (2016) 0.79

Complement component C3 - The "Swiss Army Knife" of innate immunity and host defense. Immunol Rev (2016) 0.78

Targeting the complement system for the management of retinal inflammatory and degenerative diseases. Eur J Pharmacol (2016) 0.78

Complement Evasion by Pathogenic Leptospira. Front Immunol (2016) 0.78

Expanding the therapeutic options for renal involvement in lupus: eculizumab, available evidence. Rheumatol Int (2017) 0.76

High-Fat Diet-Induced Complement Activation Mediates Intestinal Inflammation and Neoplasia, Independent of Obesity. Mol Cancer Res (2016) 0.75

Utilizing complement evasion strategies to design complement-based antibacterial immunotherapeutics: Lessons from the pathogenic Neisseriae. Immunobiology (2016) 0.75

Complement C3-Targeted Therapy: Replacing Long-Held Assertions with Evidence-Based Discovery. Trends Immunol (2017) 0.75

Monitoring of complement activation biomarkers and eculizumab in complement-mediated renal disorders. Clin Exp Immunol (2016) 0.75

A previously unrecognized role of C3a in proteinuric progressive nephropathy. Sci Rep (2016) 0.75

Complement: a key system for immune surveillance and homeostasis. Nat Immunol (2010) 10.37

Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A (1990) 7.19

Soluble human complement receptor type 1: in vivo inhibitor of complement suppressing post-ischemic myocardial inflammation and necrosis. Science (1990) 4.67

Deficiency of the GPI anchor caused by a somatic mutation of the PIG-A gene in paroxysmal nocturnal hemoglobinuria. Cell (1993) 4.04

Acquired and genetic complement abnormalities play a critical role in dense deposit disease and other C3 glomerulopathies. Kidney Int (2012) 3.99

Intra-cerebral injection of neuromyelitis optica immunoglobulin G and human complement produces neuromyelitis optica lesions in mice. Brain (2010) 3.95

Circulating complement activation in patients with anti-neutrophil cytoplasmic antibody-associated vasculitis. Kidney Int (2012) 3.34

AQP4 antibodies in neuromyelitis optica: diagnostic and pathogenetic relevance. Nat Rev Neurol (2010) 3.28

C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up. Kidney Int (2012) 3.05

The role of the anaphylatoxins in health and disease. Mol Immunol (2009) 2.95

C3 glomerulopathy: consensus report. Kidney Int (2013) 2.85

Eculizumab for dense deposit disease and C3 glomerulonephritis. Clin J Am Soc Nephrol (2012) 2.83

Multicenter phase 3 study of the complement inhibitor eculizumab for the treatment of patients with paroxysmal nocturnal hemoglobinuria. Blood (2007) 2.76

Aquaporin 4 and neuromyelitis optica. Lancet Neurol (2012) 2.58

Eculizumab in AQP4-IgG-positive relapsing neuromyelitis optica spectrum disorders: an open-label pilot study. Lancet Neurol (2013) 2.46

Predominant role for C5b-9 in renal ischemia/reperfusion injury. J Clin Invest (2000) 2.45

Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies. Am J Pathol (2007) 2.41

Effect of the complement inhibitor eculizumab on thromboembolism in patients with paroxysmal nocturnal hemoglobinuria. Blood (2007) 2.36

Function, structure and therapeutic potential of complement C5a receptors. Br J Pharmacol (2007) 2.25

Shiga toxins--from cell biology to biomedical applications. Nat Rev Microbiol (2009) 2.19

Membrane complement regulatory proteins. Clin Immunol (2005) 2.13

Complement in immune and inflammatory disorders: pathophysiological mechanisms. J Immunol (2013) 2.10

Lifetime course of myasthenia gravis. Muscle Nerve (2008) 2.07

Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol (2014) 2.06

Properdin can initiate complement activation by binding specific target surfaces and providing a platform for de novo convertase assembly. J Immunol (2007) 2.00

Terminal complement inhibition decreases antibody-mediated rejection in sensitized renal transplant recipients. Am J Transplant (2011) 1.98

C5a receptor mediates neutrophil activation and ANCA-induced glomerulonephritis. J Am Soc Nephrol (2008) 1.92

Genetic variants in C5 and poor response to eculizumab. N Engl J Med (2014) 1.91

TNT003, an inhibitor of the serine protease C1s, prevents complement activation induced by cold agglutinins. Blood (2014) 1.90

STEC-HUS, atypical HUS and TTP are all diseases of complement activation. Nat Rev Nephrol (2012) 1.89

Inhibition of human complement by a C3-binding peptide isolated from a phage-displayed random peptide library. J Immunol (1996) 1.86

Efficacy and safety of eculizumab in atypical hemolytic uremic syndrome from 2-year extensions of phase 2 studies. Kidney Int (2015) 1.85

Design and development of TT30, a novel C3d-targeted C3/C5 convertase inhibitor for treatment of human complement alternative pathway-mediated diseases. Blood (2011) 1.82

Inhibiting the C5-C5a receptor axis. Mol Immunol (2011) 1.73

Complement factor C5a mediates renal ischemia-reperfusion injury independent from neutrophils. J Immunol (2003) 1.71

Complement regulator CD46 temporally regulates cytokine production by conventional and unconventional T cells. Nat Immunol (2010) 1.67

Complement fraction 3 binding on erythrocytes as additional mechanism of disease in paroxysmal nocturnal hemoglobinuria patients treated by eculizumab. Blood (2009) 1.59

Complement inhibition enables renal allograft accommodation and long-term engraftment in presensitized nonhuman primates. Am J Transplant (2011) 1.57

Peptide inhibitors of C3 activation as a novel strategy of complement inhibition for the treatment of paroxysmal nocturnal hemoglobinuria. Blood (2014) 1.55

Treatment of neuromyelitis optica: state-of-the-art and emerging therapies. Nat Rev Neurol (2014) 1.53

Complement System Part I - Molecular Mechanisms of Activation and Regulation. Front Immunol (2015) 1.52

Inherited deficiency of mannan-binding lectin-associated serine protease 2. N Engl J Med (2003) 1.52

Complement C3dg-mediated erythrophagocytosis: implications for paroxysmal nocturnal hemoglobinuria. Blood (2015) 1.50

Autoantibodies to lipoprotein-related protein 4 in patients with double-seronegative myasthenia gravis. Arch Neurol (2011) 1.47

Myasthenia gravis: an update for the clinician. Clin Exp Immunol (2014) 1.45

Pathology after eculizumab in dense deposit disease and C3 GN. J Am Soc Nephrol (2012) 1.43

Complement activation is involved in renal damage in human antineutrophil cytoplasmic autoantibody associated pauci-immune vasculitis. J Clin Immunol (2008) 1.42

New analogs of the clinical complement inhibitor compstatin with subnanomolar affinity and enhanced pharmacokinetic properties. Immunobiology (2012) 1.39

Local extravascular pool of C3 is a determinant of postischemic acute renal failure. FASEB J (2006) 1.38

Incidence of dermatomyositis and clinically amyopathic dermatomyositis: a population-based study in Olmsted County, Minnesota. Arch Dermatol (2010) 1.35

Immunotherapy of myositis: issues, concerns and future prospects. Nat Rev Rheumatol (2010) 1.35

The use of antibody to complement protein C5 for salvage treatment of severe antibody-mediated rejection. Am J Transplant (2008) 1.34

Complement factor D, a novel serine protease. Protein Sci (1996) 1.33

Complement alternative pathway acts as a positive feedback amplification of neutrophil activation. Blood (2010) 1.31

Complement--tapping into new sites and effector systems. Nat Rev Immunol (2014) 1.30

Regulation of humoral immunity by complement. Immunity (2012) 1.30

The role of complement in antibody-mediated rejection in kidney transplantation. Nat Rev Nephrol (2012) 1.29

The complement system in B cell regulation. Mol Immunol (2004) 1.28

The role of complement in the early immune response to transplantation. Nat Rev Immunol (2012) 1.26

International Union of Basic and Clinical Pharmacology. [corrected]. LXXXVII. Complement peptide C5a, C4a, and C3a receptors. Pharmacol Rev (2013) 1.22

The complement receptor 2/factor H fusion protein TT30 protects paroxysmal nocturnal hemoglobinuria erythrocytes from complement-mediated hemolysis and C3 fragment. Blood (2012) 1.21

The receptor for complement component C3a mediates protection from intestinal ischemia-reperfusion injuries by inhibiting neutrophil mobilization. Proc Natl Acad Sci U S A (2013) 1.21

Key advances in the clinical approach to ANCA-associated vasculitis. Nat Rev Rheumatol (2014) 1.21

Long-lasting treatment effect of rituximab in MuSK myasthenia. Neurology (2012) 1.19

Cold agglutinin disease. Blood (2013) 1.18

HAE update: epidemiology and burden of disease. Allergy Asthma Proc (2013) 1.16

Mannan-binding lectin deficiency - Good news, bad news, doesn't matter? Clin Immunol (2011) 1.15

Complement factor D in age-related macular degeneration. Invest Ophthalmol Vis Sci (2011) 1.14

A phase I study of recombinant human C1 inhibitor in asymptomatic patients with hereditary angioedema. J Allergy Clin Immunol (2005) 1.14

Demographic and clinical features of neuromyelitis optica: A review. Mult Scler (2015) 1.14

Systemic complement inhibition with eculizumab for geographic atrophy in age-related macular degeneration: the COMPLETE study. Ophthalmology (2013) 1.12

Antigen-presenting cell-derived complement modulates graft-versus-host disease. J Clin Invest (2012) 1.09

Compstatin: a C3-targeted complement inhibitor reaching its prime for bedside intervention. Eur J Clin Invest (2015) 1.08

Eculizumab in atypical hemolytic-uremic syndrome. N Engl J Med (2013) 1.08

Rational engineering of a minimized immune inhibitor with unique triple-targeting properties. J Immunol (2013) 1.07

Paroxysmal nocturnal hemoglobinuria and the complement system: recent insights and novel anticomplement strategies. Adv Exp Med Biol (2013) 1.04

Polymorphism of the complement receptor 1 gene correlates with the hematologic response to eculizumab in patients with paroxysmal nocturnal hemoglobinuria. Haematologica (2013) 1.04

Soluble CR1 therapy improves complement regulation in C3 glomerulopathy. J Am Soc Nephrol (2013) 1.04

A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis. Muscle Nerve (2013) 1.03

p38MAPK, ERK and PI3K signaling pathways are involved in C5a-primed neutrophils for ANCA-mediated activation. PLoS One (2012) 1.01

Contribution of renal secreted complement C3 to the circulating pool in humans. J Immunol (1999) 1.01

Deficient alternative complement pathway activation due to factor D deficiency by 2 novel mutations in the complement factor D gene in a family with meningococcal infections. Blood (2006) 1.01

Inhibiting alternative pathway complement activation by targeting the factor D exosite. J Biol Chem (2012) 1.01

IVIG blocks complement deposition mediated by anti-GM1 antibodies in multifocal motor neuropathy. J Neurol Neurosurg Psychiatry (2010) 1.00

A family with complement factor D deficiency. J Clin Invest (2001) 1.00

An update in guillain-barré syndrome. Autoimmune Dis (2014) 1.00

Taking complement to the clinic--has the time finally come? Scand J Immunol (2009) 0.95

Complement in paroxysmal nocturnal hemoglobinuria: exploiting our current knowledge to improve the treatment landscape. Expert Rev Hematol (2014) 0.94

Therapeutic control of complement activation at the level of the central component C3. Immunobiology (2015) 0.94

Ornithodoros moubata complement inhibitor is an equally effective C5 inhibitor in pigs and humans. J Immunol (2011) 0.93

An open label clinical trial of complement inhibition in multifocal motor neuropathy. J Peripher Nerv Syst (2011) 0.92

The use of TP10, soluble complement receptor 1, in cardiopulmonary bypass. Expert Rev Cardiovasc Ther (2006) 0.92

Compstatin analog Cp40 inhibits complement dysregulation in vitro in C3 glomerulopathy. Immunobiology (2015) 0.90

Phylogenetic aspects of the complement system. Dev Comp Immunol (2001) 0.90

Spectrum of complement-mediated thrombotic microangiopathies: pathogenetic insights identifying novel treatment approaches. Semin Thromb Hemost (2014) 0.90

A phase ia dose-escalation study of the anti-factor D monoclonal antibody fragment FCFD4514S in patients with geographic atrophy. Retina (2014) 0.89

Novel roles for complement receptors in T cell regulation and beyond. Mol Immunol (2013) 0.89

Change in drusen volume as a novel clinical trial endpoint for the study of complement inhibition in age-related macular degeneration. Ophthalmic Surg Lasers Imaging Retina (2014) 0.89

Red blood cell destruction in autoimmune hemolytic anemia: role of complement and potential new targets for therapy. Biomed Res Int (2015) 0.88

Myasthenia gravis and related disorders: Pathology and molecular pathogenesis. Biochim Biophys Acta (2014) 0.88

Protection of host cells by complement regulators. Immunol Rev (2016) 0.79

Using an in vitro xenoantibody-mediated complement-dependent cytotoxicity model to evaluate the complement inhibitory activity of the peptidic C3 inhibitor Cp40. Clin Immunol (2015) 0.78

Complement component C3 - The "Swiss Army Knife" of innate immunity and host defense. Immunol Rev (2016) 0.78

Selectivity of C3-opsonin targeted complement inhibitors: A distinct advantage in the protection of erythrocytes from paroxysmal nocturnal hemoglobinuria patients. Immunobiology (2016) 0.78

Characterization of the first fully human anti-TEM1 scFv in models of solid tumor imaging and immunotoxin-based therapy. Cancer Immunol Immunother (2016) 0.75

Local endothelial complement activation reverses endothelial quiescence, enabling t-cell homing, and tumor control during t-cell immunotherapy. Oncoimmunology (2017) 0.75