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

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

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

http://www.wikidata.org/entity/Q33369509

about

Relative role of genetic complement abnormalities in sporadic and familial aHUS and their impact on clinical phenotype Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome Alternative Pathway Dysregulation and the Conundrum of Complement Activation by IgG4 Immune Complexes in Membranous Nephropathy Genetic variants in the complement system predisposing to age-related macular degeneration: a review Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome.Complement in glomerular injury.Mutations of factor H impair regulation of surface-bound C3b by three mechanisms in atypical hemolytic uremic syndrome.Genetics and complement in atypical HUS.Binding of the human complement regulators CFHR1 and factor H by streptococcal collagen-like protein 1 (Scl1) via their conserved C termini allows control of the complement cascade at multiple levels.Advances in our understanding of the pathogenesis of glomerular thrombotic microangiopathy.Disease-associated N-terminal complement factor H mutations perturb cofactor and decay-accelerating activities.The spectrum of renal thrombotic microangiopathy in lupus nephritis.Tissue-specific host recognition by complement factor H is mediated by differential activities of its glycosaminoglycan-binding regions.Factors determining penetrance in familial atypical haemolytic uraemic syndrome.A 'silent', new polymorphism of factor H and apparent de novo atypical haemolytic uraemic syndrome after kidney transplantation 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.Plasma exchange in Immunoglobulin A nephropathy with thrombotic microangiopathy and acute cortical necrosis.Smallpox inhibitor of complement enzymes (SPICE): dissecting functional sites and abrogating activity Whole-exome sequencing identifies rare, functional CFH variants in families with macular degeneration.Atypical hemolytic uremic syndrome and mutation analysis of factor H gene in two Tunisian families.Analysis of rare variants in the CFH gene in patients with the cuticular drusen subtype of age-related macular degeneration A rare penetrant mutation in CFH confers high risk of age-related macular degeneration.Mapping rare, deleterious mutations in Factor H: Association with early onset, drusen burden, and lower antigenic levels in familial AMD.Complement Dysregulation and Disease: Insights from Contemporary Genetics.Rare Variants in the Functional Domains of Complement Factor H Are Associated With Age-Related Macular Degeneration.The Genetics of Ultra-Rare Renal Disease.Translational mini-review series on complement factor H: structural and functional correlations for factor H.The C-terminus of complement factor H is essential for host cell protection.Identification of a rare coding variant in complement 3 associated with age-related macular degeneration Rare variants in CFI, C3 and C9 are associated with high risk of advanced age-related macular degeneration.Factor h: a complement regulator in health and disease, and a mediator of cellular interactions.Glomerular basement membrane heparan sulfate in health and disease: A regulator of local complement activation.The complement system in age-related macular degeneration: A review of rare genetic variants and implications for personalized treatment.Complement regulator Factor H mediates a two-step uptake of Streptococcus pneumoniae by human cells.Complement factor H R1210C among Japanese patients with age-related macular degeneration.Complement Factor H-Related 5-Hybrid Proteins Anchor Properdin and Activate Complement at Self-Surfaces.Disease-linked mutations in factor H reveal pivotal role of cofactor activity in self-surface-selective regulation of complement activation.Identification of factor H-like protein 1 as the predominant complement regulator in Bruch's membrane: implications for age-related macular degeneration.The Murine Factor H-Related Protein FHR-B Promotes Complement Activation Role of Factor H and Related Proteins in Regulating Complement Activation in the Macula, and Relevance to Age-Related Macular Degeneration.

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P2860

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

http://www.wikidata.org/entity/Q33369509

description

2005 nî lūn-bûn

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2005 թուականի Դեկտեմբերին հրատարակուած գիտական յօդուած

@hyw

2005 թվականի դեկտեմբերին հրատարակված գիտական հոդված

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2005年の論文

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2005年論文

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2005年論文

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2005年論文

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2005年論文

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2005年論文

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2005年论文

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name

Factor H and atypical hemolyti ...... fective recognition functions.

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Factor H and atypical hemolyti ...... fective recognition functions.

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Factor H and atypical hemolyti ...... fective recognition functions.

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Factor H and atypical hemolyti ...... fective recognition functions.

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Factor H and atypical hemolyti ...... fective recognition functions.

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Journal of the American Society of Nephrology

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Factor H and atypical hemolyti ...... fective recognition functions.

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Andrea Hartmann

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Anja Kunert

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Christine Skerka

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Christoph Licht

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Clemens W Ostrowicz

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Heiko Richter

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Rebecca E Saunders

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Steffi Hälbich

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Stephen J Perkins

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170-177

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scholarly article

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10.1681/ASN.2005080868

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1

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17

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16338962

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