期刊
KIDNEY INTERNATIONAL
卷 80, 期 4, 页码 397-404出版社
NATURE PUBLISHING GROUP
DOI: 10.1038/ki.2011.152
关键词
autoantibody; complement; factor H; factor H-related proteins; hemolytic uremic syndrome
资金
- Deutsche Forschungsgemeinschaft [JO 144/1-1]
- Spanish Ministerio de Ciencia e Innovacion [FIS 06/0625, PS 09/00268]
- 'Fundacion para la Investigacion Biomedica Hospital Universitario La Paz' (FIBHULP)
- 'Centro de Investigacion Biomedica en Red de Enfermedades Raras' (CIBERER)
The autoimmune form of atypical hemolytic uremic syndrome (HUS) is characterized by circulating autoantibodies against the complement regulator factor H, and is often associated with deficiency of the factor H-related proteins CFHR1 and CFHR3. Here we studied whether anti-factor H autoantibodies crossreact with CFHR1, and determined functional consequences of this. In ELISA, anti-factor H immunoglobulin G (IgG) autoantibodies from 24 atypical HUS patients bound to the short consensus repeat 20 domain of factor H, 21 antibodies also recognized CFHR1, but none CFHR3. Three patients also had anti-factor H IgA autoantibodies crossreacting with CFHR1. Analysis of the IgG fractions in CFHR1-deficient patients found that CFHR1-IgG complexes were formed during plasma exchange treatment, indicating that autoantibodies recognize CFHR1 in vivo. Recombinant CFHR1 prevented hemolysis of sheep erythrocytes caused by patient plasma containing anti-factor H IgG, but it did not inhibit red cell lysis caused by a factor H mutation (W1183 L) in the short consensus repeat 20 domain. Thus, exogenous CFHR1 provided during plasma exchange therapy may neutralize anti-factor H autoantibodies and help in the treatment of autoimmune atypical HUS. Kidney International (2011) 80, 397-404; doi:10.1038/ki.2011.152; published online 15 June 2011
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