Human C3 mutation reveals a mechanism of dense deposit disease pathogenesis and provides insights into complement activation and regulation (Q34162206)

31 July 2017

title

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

1 reference

Santiago Rodriguez de Córdoba

31 July 2017

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13

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31 July 2017

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Rubén Martínez-Barricarte

1

1 reference

31 July 2017

Meike Heurich

2

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31 July 2017

Francisco Valdes-Cañedo

3

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31 July 2017

Eduardo Vazquez-Martul

4

1 reference

31 July 2017

Eva Torreira

5

1 reference

31 July 2017

Tamara Montes

6

1 reference

31 July 2017

Agustín Tortajada

7

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31 July 2017

Sheila Pinto

8

1 reference

31 July 2017

Margarita Lopez-Trascasa

9

1 reference

31 July 2017

B Paul Morgan

10

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31 July 2017

Oscar Llorca

11

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31 July 2017

Claire L Harris

12

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31 July 2017

language of work or name

English

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publication date

13 September 2010

1 reference

Journal of Clinical Investigation

31 July 2017

published in

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31 July 2017

volume

120

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31 July 2017

issue

10

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31 July 2017

page(s)

3702-3712

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

31 July 2017

cites work

1 reference

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

5 July 2018

1 reference

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

5 July 2018

1 reference

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

5 July 2018

1 reference

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Structural and Functional Analysis of a C3b-specific Antibody That Selectively Inhibits the Alternative Pathway of Complement

5 July 2018

1 reference

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Interaction between the coagulation and complement system

5 July 2018

1 reference

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Measurement of factor H variants in plasma using variant-specific monoclonal antibodies: application to assessing risk of age-related macular degeneration

5 July 2018

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

5 July 2018

1 reference

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The tick-over theory revisited: formation and regulation of the soluble alternative complement C3 convertase (C3(H2O)Bb)

5 July 2018

1 reference

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Translational mini-review series on complement factor H: genetics and disease associations of human complement factor H.

5 July 2018

1 reference

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Complement driven by conformational changes

5 July 2018

1 reference

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Structure of compstatin in complex with complement component C3c reveals a new mechanism of complement inhibition

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

New approaches to the treatment of dense deposit disease

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Dense deposit disease: new insights

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

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

5 July 2018

1 reference

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Structure of C3b in complex with CRIg gives insights into regulation of complement activation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Modeling how CD46 deficiency predisposes to atypical hemolytic uremic syndrome

5 July 2018

1 reference

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Implications of the initial mutations in membrane cofactor protein (MCP; CD46) leading to atypical hemolytic uremic syndrome

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Prevention of C5 activation ameliorates spontaneous and experimental glomerulonephritis in factor H-deficient mice

5 July 2018

1 reference

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Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II)

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Structures of complement component C3 provide insights into the function and evolution of immunity

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Complement and diseases: defective alternative pathway control results in kidney and eye diseases

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Heterozygous and homozygous factor h deficiencies associated with hemolytic uremic syndrome or membranoproliferative glomerulonephritis: report and genetic analysis of 16 cases

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

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

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Complement factor I deficiency associated with recurrent meningitis coinciding with menstruation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Complement. Second of two parts

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Complement. First of two parts

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Human factor H deficiency. Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Mapping of the complement regulatory domains in the human factor H-like protein 1 and in factor H1.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

The alternative pathway C3/C5 convertase: chemical basis of factor B activation

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

The amplification loop of the complement pathways.

5 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Molecular dissection of interactions between components of the alternative pathway of complement and decay accelerating factor (CD55).

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H

28 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2947238

A new screening test for C3 nephritis factor based on a stable cell bound convertase on sheep erythrocytes

28 October 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20852386

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identification of complement regulatory domains in human factor H

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/20852386

inferred from PubMed ID database lookup

12 December 2020

based on heuristic

Identifiers

DOI

10.1172/JCI43343

1 reference

31 July 2017

1 reference

31 July 2017

1 reference