DEAP-HUS: deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome (Q33388274)

26 July 2017

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title

DEAP-HUS: deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome (English)

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26 July 2017

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1

Peter F Zipfel

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26 July 2017

6

Christine Skerka

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26 July 2017

4

Christoph Licht

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26 July 2017

Christoph Mache

2

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26 July 2017

Dominik Müller

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3

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26 July 2017

Marianne Wigger

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5

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26 July 2017

European DEAP-HUS Study Group

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7

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26 July 2017

16 February 2010

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26 July 2017

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26 July 2017

25

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10

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26 July 2017

2009-2019

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https://scigraph.springernature.com/pub.10.1007/s00467-010-1446-9

26 July 2017

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cites work

The C-terminus of factor H: monoclonal antibodies inhibit heparin binding and identify epitopes common to factor H and factor H-related proteins

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

21 January 2018

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Human factor H-related protein 5 has cofactor activity, inhibits C3 convertase activity, binds heparin and C-reactive protein, and associates with lipoprotein.

21 January 2018

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https://api.crossref.org/works/10.1007%2FS00467-010-1446-9

21 January 2018

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12 December 2020

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

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Varicella as a trigger of atypical haemolytic uraemic syndrome associated with complement dysfunction: two cases

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Autoimmune forms of thrombotic microangiopathy and membranoproliferative glomerulonephritis: Indications for a disease spectrum and common pathogenic principles

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Eculizumab for Atypical Hemolytic–Uremic Syndrome

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The human factor H-related protein 4 (FHR-4). A novel short consensus repeat-containing protein is associated with human triglyceride-rich lipoproteins

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12 December 2020

Deletion of complement factor H-related genes CFHR1 and CFHR3 is associated with atypical hemolytic uremic syndrome

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ADAMTS13 autoantibodies in patients with thrombotic microangiopathies and other immunomediated diseases

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12 December 2020

Molecular cloning of a human serum protein structurally related to complement factor H

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Atypical haemolytic uraemic syndrome associated with a hybrid complement gene

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12 December 2020

The high frequency of complement factor H related CFHR1 gene deletion is restricted to specific subgroups of patients with atypical haemolytic uraemic syndrome.

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Rituximab as pre-emptive treatment in patients with thrombotic thrombocytopenic purpura and evidence of anti-ADAMTS13 autoantibodies

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Complement regulators and inhibitory proteins

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Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome.

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12 December 2020

Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome

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Complement inhibitor eculizumab in atypical hemolytic uremic syndrome

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12 December 2020

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

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12 December 2020

Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency

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Binding of human factor H-related protein 1 to serum-resistant Borrelia burgdorferi is mediated by borrelial complement regulator-acquiring surface proteins

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Factor H family proteins and human diseases

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FHL-1/reconectin: a human complement and immune regulator with cell-adhesive function

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Autoantibodies in haemolytic uraemic syndrome (HUS).

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Coagulation factor XIII B subunit is encoded by a gene linked to the regulator of complement activation (RCA) gene cluster in man

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Guideline for the investigation and initial therapy of diarrhea-negative hemolytic uremic syndrome.

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The complement factor H R1210C mutation is associated with atypical hemolytic uremic syndrome

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Successful pre-transplant management of a patient with anti-factor H autoantibodies-associated haemolytic uraemic syndrome

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A novel short consensus repeat-containing molecule is related to human complement factor H

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12 December 2020

Factor H and atypical hemolytic uremic syndrome: mutations in the C-terminus cause structural changes and defective recognition functions

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The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20.

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Functional properties of complement factor H-related proteins FHR-3 and FHR-4: binding to the C3d region of C3b and differential regulation by heparin

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Mechanisms of killing by anti-CD20 monoclonal antibodies.

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12 December 2020

Hemolytic Uremic Syndrome

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12 December 2020

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

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12 December 2020

Two additional human serum proteins structurally related to complement factor H. Evidence for a family of factor H-related genes.

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12 December 2020

Atypical hemolytic uremic syndrome associated with complement factor H autoantibodies and CFHR1/CFHR3 deficiency

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12 December 2020

The C-terminus of complement regulator Factor H mediates target recognition: evidence for a compact conformation of the native protein

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12 December 2020

Complement and the atypical hemolytic uremic syndrome in children.

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12 December 2020

Thrombomodulin mutations in atypical hemolytic-uremic syndrome

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12 December 2020

Structural and functional characterization of factor H mutations associated with atypical hemolytic uremic syndrome

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12 December 2020

The interactive Factor H-atypical hemolytic uremic syndrome mutation database and website: update and integration of membrane cofactor protein and Factor I mutations with structural models

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12 December 2020

FHR-4A: a new factor H-related protein is encoded by the human FHR-4 gene.

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12 December 2020

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

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12 December 2020

The role of complement inhibition in PNH.

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12 December 2020

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

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12 December 2020

10.1007/S00467-010-1446-9

Identifiers

DOI

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Dimensions Publication ID

26 July 2017

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