Mutations of factor H impair regulation of surface-bound C3b by three mechanisms in atypical hemolytic uremic syndrome. - Wikidata - awgldk - TriplyDB

Mutations of factor H impair regulation of surface-bound C3b by three mechanisms in atypical hemolytic uremic syndrome.

Mutations of factor H impair regulation of surface-bound C3b by three mechanisms in atypical hemolytic uremic syndrome.

http://www.wikidata.org/entity/Q33384039

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Structural analysis of the C-terminal region (modules 18-20) of complement regulator factor H (FH)Atypical hemolytic uremic syndrome Structural basis for engagement by complement factor H of C3b on a self surface Dual interaction of factor H with C3d and glycosaminoglycans in host-nonhost discrimination by complement Structural Basis for Complement Evasion by Lyme Disease Pathogen Borrelia burgdorferi Properdin: a tightly regulated critical inflammatory modulator Complement System Part II: Role in Immunity Genetics and complement in atypical HUS.Disease-associated N-terminal complement factor H mutations perturb cofactor and decay-accelerating activities.Crystallographic determination of the disease-associated T1184R variant of complement regulator factor H.Complement activation during liver transplantation-special emphasis on patients with atypical hemolytic uremic syndrome.Recognition of malondialdehyde-modified proteins by the C terminus of complement factor H is mediated via the polyanion binding site and impaired by mutations found in atypical hemolytic uremic syndrome.Complement factor B mutations in atypical hemolytic uremic syndrome-disease-relevant or benign?Complement activation patterns in atypical haemolytic uraemic syndrome during acute phase and in remission.Factors determining penetrance in familial atypical haemolytic uraemic syndrome.Mapping interactions between complement C3 and regulators using mutations in atypical hemolytic uremic syndrome.The major autoantibody epitope on factor H in atypical hemolytic uremic syndrome is structurally different from its homologous site in factor H-related protein 1, supporting a novel model for induction of autoimmunity in this disease.Minor Role of Plasminogen in Complement Activation on Cell Surfaces.Self-nonself discrimination by the complement system.HUS and atypical HUS.Complement control protein factor H: the good, the bad, and the inadequate.Factor h and properdin recognize different epitopes on renal tubular epithelial heparan sulfate.Complement System Part I - Molecular Mechanisms of Activation and Regulation.Microbes bind complement inhibitor factor H via a common site Association of a novel complement factor H mutation with severe crescentic and necrotizing glomerulonephritis.Secondary focal and segmental glomerulosclerosis associated with single-nucleotide polymorphisms in the genes encoding complement factor H and C3.Essential role of surface-bound complement factor H in controlling immune complex-induced arthritis Insights into the Effects of Complement Factor H on the Assembly and Decay of the Alternative Pathway C3 Proconvertase and C3 Convertase.Complementing the Sugar Code: Role of GAGs and Sialic Acid in Complement Regulation Mutations in Complement Factor H Impair Alternative Pathway Regulation on Mouse Glomerular Endothelial Cells in Vitro.Eculizumab Dosing Regimen in Atypical HUS: Possibilities for Individualized Treatment.Protection of host cells by complement regulators.Disease-linked mutations in factor H reveal pivotal role of cofactor activity in self-surface-selective regulation of complement activation.Rationale for Adjunctive Therapies for Pediatric Sepsis Induced Multiple Organ Failure.Crystal structure of a tripartite complex between C3dg, C-terminal domains of factor H and OspE of Borrelia burgdorferi.Factor H C-Terminal Domains Are Critical for Regulation of Platelet/Granulocyte Aggregate Formation.Factor H-Related (FHR)-1 and FHR-2 Form Homo- and Heterodimers, while FHR-5 Circulates Only As Homodimer in Human Plasma.Diseases of complement dysregulation-an overview.Antibody mediated rejection associated with complement factor h-related protein 3/1 deficiency successfully treated with eculizumab.A novel method for direct measurement of complement convertases activity in human serum.

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P2860

Mutations of factor H impair regulation of surface-bound C3b by three mechanisms in atypical hemolytic uremic syndrome.

http://www.wikidata.org/entity/Q33384039

description

2009 nî lūn-bûn

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2009 թուականի Ապրիլին հրատարակուած գիտական յօդուած

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2009 թվականի ապրիլին հրատարակված գիտական հոդված

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2009年の論文

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2009年論文

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2009年論文

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2009年論文

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2009年论文

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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type

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

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P1433

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Journal of Biological Chemistry

P1476

Mutations of factor H impair r ...... cal hemolytic uremic syndrome.

@en

P2093

Angelique L Rops

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David E Isenman

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Markus J Lehtinen

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T Sakari Jokiranta

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P2860

N.m.r. and molecular-modelling studies of the solution conformation of heparin

Structure of complement factor H carboxyl-terminus reveals molecular basis of atypical haemolytic uremic syndrome.

Control of the amplification convertase of complement by the plasma protein beta1H

Human complement C3b inactivator: isolation, characterization, and demonstration of an absolute requirement for the serum protein beta1H for cleavage of C3b and C4b in solution

X-ray crystal structure of C3d: a C3 fragment and ligand for complement receptor 2

Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura

A new map of glycosaminoglycan and C3b binding sites on factor H

Disease-associated sequence variations congregate in a polyanion recognition patch on human factor H revealed in three-dimensional structure

Comparison of surface recognition and C3b binding properties of mouse and human complement factor H

Interaction between the third complement protein and cell surface macromolecules

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15650-15658

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scholarly article

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10.1074/JBC.M900814200

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23

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284

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Johan Van Der Vlag

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2009-04-07T00:00:00Z

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19351878

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2708861

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