STEC-HUS, atypical HUS and TTP are all diseases of complement activation (Q33403359)

26 July 2017

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

1 reference

26 July 2017

Giuseppe Remuzzi

3

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author name string

Marina Noris

series ordinal

1

1 reference

PubMed ID

22986360

26 July 2017

Federica Mescia

series ordinal

2

1 reference

PubMed ID

22986360

26 July 2017

language of work or name

English

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publication date

18 September 2012

1 reference

Nature Reviews Nephrology

26 July 2017

1 reference

26 July 2017

8

1 reference

26 July 2017

11

1 reference

26 July 2017

622-633

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https://scigraph.springernature.com/pub.10.1038/nrneph.2012.195

26 July 2017

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cites work

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura

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von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome

21 January 2018

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HUS and TTP: variable expression of a single entity

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Atypical hemolytic-uremic syndrome

21 January 2018

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Genetics and complement in atypical HUS.

21 January 2018

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https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome

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Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura

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Pathophysiology of thrombotic thrombocytopenic purpura

21 January 2018

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Is severe deficiency of ADAMTS-13 specific for thrombotic thrombocytopenic purpura? Yes

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21 January 2018

The complement system

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Complement: a key system for immune surveillance and homeostasis

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Alternative pathway activation of complement by Shiga toxin promotes exuberant C3a formation that triggers microvascular thrombosis

21 January 2018

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The cytolytically inactive terminal complement complex activates endothelial cells to express adhesion molecules and tissue factor procoagulant activity

21 January 2018

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Release of heparan sulfate from endothelial cells. Implications for pathogenesis of hyperacute rejection

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Transient perturbation of endothelial integrity induced by natural antibodies and complement

21 January 2018

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C5a-induced gene expression in human umbilical vein endothelial cells

21 January 2018

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Cytolytically inactive terminal complement complex causes transendothelial migration of polymorphonuclear leukocytes in vitro and in vivo

21 January 2018

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Mechanisms of signal transduction activated by sublytic assembly of terminal complement complexes on nucleated cells

21 January 2018

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The role of the anaphylatoxins in health and disease

21 January 2018

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The human complement system in thrombin-mediated platelet function

21 January 2018

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Human platelet activation by C3a and C3a des-arg

21 January 2018

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Factor H dysfunction in patients with atypical hemolytic uremic syndrome contributes to complement deposition on platelets and their activation

21 January 2018

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Abnormal platelet function in C3-deficient mice.

21 January 2018

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Complement activation on platelets: implications for vascular inflammation and thrombosis

21 January 2018

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Molecular intercommunication between the complement and coagulation systems

21 January 2018

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https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Epidemic profile of Shiga-toxin-producing Escherichia coli O104:H4 outbreak in Germany

21 January 2018

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https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Origins of the E. coli strain causing an outbreak of hemolytic-uremic syndrome in Germany

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Shiga toxin-2 triggers endothelial leukocyte adhesion and transmigration via NF-kappaB dependent up-regulation of IL-8 and MCP-1.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

The CXCR4/CXCR7/SDF-1 pathway contributes to the pathogenesis of Shiga toxin-associated hemolytic uremic syndrome in humans and mice

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Verotoxin-1-induced up-regulation of adhesive molecules renders microvascular endothelial cells thrombogenic at high shear stress

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Shiga toxin enhances functional tissue factor on human glomerular endothelial cells: implications for the pathophysiology of hemolytic uremic syndrome

21 January 2018

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https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Platelet activation by Shiga toxin and circulatory factors as a pathogenetic mechanism in the hemolytic uremic syndrome.

21 January 2018

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https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Letter: Plasma-C3 in haemolytic-uraemic syndrome and thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Letter: Serum-complement levels in haemolytic-uraemic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab in severe Shiga-toxin-associated HUS

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Alternative pathway of complement in children with diarrhea-associated hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement activation on platelet-leukocyte complexes and microparticles in enterohemorrhagic Escherichia coli-induced hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Platelet activation leads to activation and propagation of the complement system

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Shiga toxin activates complement and binds factor H: evidence for an active role of complement in hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

The association between idiopathic hemolytic uremic syndrome and infection by verotoxin-producing Escherichia coli

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Hemolytic-uremic syndrome and Vero cytotoxin-producing Escherichia coli infection in Italy. The HUS Italian Study Group

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Haemolytic uraemic syndrome and mutations of the factor H gene: a registry-based study of German speaking countries

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host cell recognition

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Familial haemolytic uraemic syndrome and an MCP mutation

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Mutations in human complement regulator, membrane cofactor protein (CD46), predispose to development of familial hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Mutations in complement factor I predispose to development of atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Thrombomodulin mutations in atypical hemolytic-uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Anti-Factor H autoantibodies associated with atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Clinical features of anti-factor H autoantibody-associated hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

The binding of factor H to a complex of physiological polyanions and C3b on cells is impaired in atypical hemolytic uremic syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Mutations in factor H reduce binding affinity to C3b and heparin and surface attachment to endothelial cells in hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Hemolytic uremic syndrome: a factor H mutation (E1172Stop) causes defective complement control at the surface of endothelial cells

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Functional analyses indicate a pathogenic role of factor H autoantibodies in atypical haemolytic uraemic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Genetic and functional analyses of membrane cofactor protein (CD46) mutations in atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Characterization of mutations in complement factor I (CFI) associated with hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Familial hemolytic uremic syndrome associated with complement factor H deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Familial, recurrent haemolytic-uraemic syndrome with hypocomplementaemia

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Phenotypic expression of factor H mutations in patients with atypical hemolytic uremic syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Association of factor H autoantibodies with deletions of CFHR1, CFHR3, CFHR4, and with mutations in CFH, CFI, CD46, and C3 in patients with atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Insights into hemolytic uremic syndrome: segregation of three independent predisposition factors in a large, multiple affected pedigree

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Predisposition to atypical hemolytic uremic syndrome involves the concurrence of different susceptibility alleles in the regulators of complement activation gene cluster in 1q32.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Spontaneous hemolytic uremic syndrome triggered by complement factor H lacking surface recognition domains

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Pregnancy-associated hemolytic uremic syndrome revisited in the era of complement gene mutations

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Membrane cofactor protein mutations in atypical hemolytic uremic syndrome (aHUS), fatal Stx-HUS, C3 glomerulonephritis, and the HELLP syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Cloning, expression, and functional characterization of the von Willebrand factor-cleaving protease (ADAMTS13).

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

ADAMTS13 activity in thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: relation to presenting features and clinical outcomes in a prospective cohort of 142 patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Specific von Willebrand factor-cleaving protease in thrombotic microangiopathies: a study of 111 cases

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Thrombotic Thrombocytopenic Purpura with C′3Vascular Deposits: Report of a Case

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Characterization of platelet glycoproteins and platelet/endothelial cell antibodies in patients with thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement activation in thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab for congenital atypical hemolytic-uremic syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab in the treatment of atypical hemolytic uremic syndrome in infants

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab: safety and efficacy after 17 months of treatment in a renal transplant patient with recurrent atypical hemolytic-uremic syndrome: case report

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Prophylactic eculizumab after renal transplantation in atypical hemolytic-uremic syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Prophylactic eculizumab prior to kidney transplantation for atypical hemolytic uremic syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Inhibiting the C5-C5a receptor axis

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Therapeutic targeting of the complement system in age-related macular degeneration: a review.

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

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

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Genetics and genetic testing in hemolytic uremic syndrome/thrombotic thrombocytopenic purpura

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Mutations in complement regulatory proteins predispose to preeclampsia: a genetic analysis of the PROMISSE cohort

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement Mutation-AssociatedDe NovoThrombotic Microangiopathy Following Kidney Transplantation

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab and renal transplantation in a patient with CAPS

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Pexelizumab in ischemic heart disease: a systematic review and meta-analysis on 15,196 patients

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Blocking the receptor for C5a in patients with rheumatoid arthritis does not reduce synovial inflammation

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab for Atypical Hemolytic–Uremic Syndrome

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Generation of C5a in the absence of C3: a new complement activation pathway

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Neutrophil adhesion to human endothelial cells is induced by the membrane attack complex: the roles of P-selectin and platelet activating factor

21 January 2018

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement activation by myeloperoxidase products released from stimulated human polymorphonuclear leukocytes

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

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

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Shiga toxins--from cell biology to biomedical applications

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Letter: Serum-complement levels in haemolytic-uraemic syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Hypocomplementemia and leukocytosis in diarrhea-associated hemolytic uremic syndrome

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Letter: Haemolytic-uraemic syndrome: evidence for intravascular C3 activation

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Complement activation: the missing link between ADAMTS-13 deficiency and microvascular thrombosis of thrombotic microangiopathies

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

Eculizumab in the treatment of refractory idiopathic thrombotic thrombocytopenic purpura

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

INF2 is another piece of the jigsaw puzzle for FSGS

31 March 2020

1 reference

https://api.crossref.org/works/10.1038%2FNRNEPH.2012.195

C5a induces tissue factor activity on endothelial cells

31 March 2020

1 reference

12 December 2020

Soluble human complement receptor 1 limits ischemic damage in cardiac surgery patients at high risk requiring cardiopulmonary bypass.

1 reference

12 December 2020

Shiga-toxin-producing Escherichia coli and haemolytic uraemic syndrome

1 reference

12 December 2020

Familial hypocomplementemic hemolytic uremic syndrome with HLA-A3,B7 haplotype

1 reference

12 December 2020

Genetic studies into inherited and sporadic hemolytic uremic syndrome

1 reference

12 December 2020

The complement system in hemolytic-uremic syndrome in childhood

1 reference

12 December 2020

In-vitro and in-vivo consequences of mutations in the von Willebrand factor cleaving protease ADAMTS13 in thrombotic thrombocytopenic purpura

1 reference

12 December 2020

Verotoxin-1 promotes leukocyte adhesion to cultured endothelial cells under physiologic flow conditions.

1 reference

12 December 2020

Human polymorphonuclear leukocytes store large amounts of terminal complement components C7 and C6, which may be released on stimulation

1 reference

12 December 2020

On the mechanism by which complement proteins C5b-9 increase platelet prothrombinase activity

1 reference

12 December 2020

Complement proteins C5b-9 cause release of membrane vesicles from the platelet surface that are enriched in the membrane receptor for coagulation factor Va and express prothrombinase activity

1 reference

12 December 2020

Effect of complement proteins C5b-9 on blood platelets. Evidence for reversible depolarization of membrane potential

1 reference

12 December 2020

Endothelial heterogeneity in Shiga toxin receptors and responses.

1 reference

12 December 2020

Biosynthesis and secretion of complement component (C3) by activated human polymorphonuclear leukocytes

1 reference

12 December 2020

Hypocomplementemia discloses genetic predisposition to hemolytic uremic syndrome and thrombotic thrombocytopenic purpura: role of factor H abnormalities. Italian Registry of Familial and Recurrent Hemolytic Uremic Syndrome/Thrombotic [...]

1 reference

12 December 2020

Effects of verocytotoxin-1 on nonadherent human monocytes: binding characteristics, protein synthesis, and induction of cytokine release.

1 reference

12 December 2020

Complement proteins C5b-9 initiate secretion of platelet storage granules without increased binding of fibrinogen or von Willebrand factor to newly expressed cell surface GPIIb-IIIa

1 reference

12 December 2020

Identifiers

DOI

10.1038/NRNEPH.2012.195

1 reference

26 July 2017

1 reference

PubMed ID

22986360