A Familial C3GN Secondary to Defective C3 Regulation by Complement Receptor 1 and Complement Factor H (Q35809292)

10 August 2017

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

1 reference

Véronique Frémeaux-Bacchi

10 August 2017

12

object named as

Veronique Fremeaux-Bacchi

1 reference

10 August 2017

Sophie Chauvet

1

1 reference

10 August 2017

Lubka T Roumenina

2

1 reference

10 August 2017

Sarah Bruneau

3

1 reference

10 August 2017

Maria Chiara Marinozzi

4

1 reference

10 August 2017

Tania Rybkine

5

1 reference

10 August 2017

Elizabeth C Schramm

6

1 reference

10 August 2017

Anuja Java

7

1 reference

10 August 2017

John P Atkinson

8

1 reference

10 August 2017

Jean Claude Aldigier

9

1 reference

10 August 2017

Frank Bridoux

10

1 reference

10 August 2017

Guy Touchard

11

1 reference

10 August 2017

language of work or name

1 reference

15 October 2015

1 reference

Journal of the American Society of Nephrology

10 August 2017

1 reference

10 August 2017

27

1 reference

10 August 2017

6

1 reference

10 August 2017

1665-1677

1 reference

Complement System Part I - Molecular Mechanisms of Activation and Regulation

10 August 2017

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Complement System Part II: Role in Immunity

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

A revised mechanism for the activation of complement C3 to C3b: a molecular explanation of a disease-associated polymorphism

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign?

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Functional evaluation of factor H genetic and acquired abnormalities: application for atypical hemolytic uremic syndrome (aHUS).

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

C3 glomerulopathy: consensus report

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Toward a working definition of C3 glomerulopathy by immunofluorescence

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Soluble CR1 therapy improves complement regulation in C3 glomerulopathy

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Autoantibodies against complement components and functional consequences.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Genetics and outcome of atypical hemolytic uremic syndrome: a nationwide French series comparing children and adults

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Use of eculizumab for atypical haemolytic uraemic syndrome and C3 glomerulopathies

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Overall neutralization of complement factor H by autoantibodies in the acute phase of the autoimmune form of atypical hemolytic uremic syndrome

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

C3 glomerulonephritis: clinicopathological findings, complement abnormalities, glomerular proteomic profile, treatment, and follow-up

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Membranoproliferative glomerulonephritis--a new look at an old entity

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structural basis for complement factor I control and its disease-associated sequence polymorphisms

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Proliferative glomerulonephritis secondary to dysfunction of the alternative pathway of complement

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Alternative complement pathway assessment in patients with atypical HUS.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structures of C3b in complex with factors B and D give insight into complement convertase formation

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structure of the Extracellular Portion of CD46 Provides Insights into Its Interactions with Complement Proteins and Pathogens

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Identification of a mutation in complement factor H-related protein 5 in patients of Cypriot origin with glomerulonephritis

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

C3 glomerulopathy: a new classification

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Coexistence of closed and open conformations of complement factor B in the alternative pathway C3bB(Mg2+) proconvertase

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structural and functional implications of the alternative complement pathway C3 convertase stabilized by a staphylococcal inhibitor

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Glomeruli of Dense Deposit Disease contain components of the alternative and terminal complement pathway

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

3D structure of the C3bB complex provides insights into the activation and regulation of the complement alternative pathway convertase.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structural transitions of complement component C3 and its activation products

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structure of C3b reveals conformational changes that underlie complement activity

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Structural insights into the central complement component C3.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Conditionally immortalized human glomerular endothelial cells expressing fenestrations in response to VEGF.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Membranoproliferative glomerulonephritis type II (dense deposit disease): an update

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Solvent accessibility of native and hydrolyzed human complement protein 3 analyzed by hydrogen/deuterium exchange and mass spectrometry.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Functional analysis in serum from atypical Hemolytic Uremic Syndrome patients reveals impaired protection of host cells associated with mutations in factor H.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

A conditionally immortalized human podocyte cell line demonstrating nephrin and podocin expression.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Identification of residues within the 727-767 segment of human complement component C3 important for its interaction with factor H and with complement receptor 1 (CR1, CD35).

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Dissection of CR1, factor H, membrane cofactor protein, and factor B binding and functional sites in the third complement component

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Immunohistochemical study of the human glomerular C3b receptor in normal kidney and in seventy-five cases of renal diseases: loss of C3b receptor antigen in focal hyalinosis and in proliferative nephritis of systemic lupus erythematosus

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Formation of the initial C3 convertase of the alternative complement pathway. Acquisition of C3b-like activities by spontaneous hydrolysis of the putative thioester in native C3

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Identification of the C3b receptor-binding domain in third component of complement

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Factor H autoantibodies in membranoproliferative glomerulonephritis.

11 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

Nucleophilic modification of human complement protein C3: correlation of conformational changes with acquisition of C3b-like functional properties

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

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

25 September 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=4884110

CRIT is expressed on podocytes in normal human kidney and upregulated in membranous nephropathy

15 November 2018

1 reference

12 December 2020

Localization of the complement regulatory proteins in the normal human kidney

1 reference

12 December 2020

Self-association and domain rearrangements between complement C3 and C3u provide insight into the activation mechanism of C3

1 reference

12 December 2020

Hypocomplementaemia of poststreptococcal acute glomerulonephritis is associated with C3 nephritic factor (C3NeF) IgG autoantibody activity

1 reference

12 December 2020

No complement receptor 1 stumps on podocytes in human glomerulopathies

1 reference

12 December 2020

Combined C3b and factor B autoantibodies and MPGN type II

1 reference

12 December 2020

Competition for binding sites on C3b by CR1, CR2, MCP, factor B and factor H

1 reference

12 December 2020

Identifiers

DOI

10.1681/ASN.2015040348

1 reference

release_ftoq6kk7efdypj4bc244nl5aeq

10 August 2017

1 reference

Fatcat

https://api.fatcat.wiki/v0/release/ftoq6kk7efdypj4bc244nl5aeq

24 November 2022

mapped directly with Wikidata item

1 reference

10 August 2017

1 reference