Characterization of genetic predisposition and autoantibody profile in atypical haemolytic-uraemic syndrome

Immunology
Bahadur Singh GurjarArvind Bagga

Abstract

We previously reported that Indian paediatric patients with atypical haemolytic-uraemic syndrome (aHUS) showed high frequencies of anti-complement factor H (FH) autoantibodies that are correlated with homozygous deletion of the genes for FH-related proteins 1 and 3 (FHR1 and FHR3) (FHR1/3-/- ). We now report that Indian paediatric aHUS patients without anti-FH autoantibodies also showed modestly higher frequencies of the FHR1/3-/- genotype. Further, when we characterized epitope specificities and binding avidities of anti-FH autoantibodies in aHUS patients, most anti-FH autoantibodies were directed towards the FH cell-surface anchoring polyanionic binding site-containing C-terminal short conservative regions (SCRs) 17-20 with higher binding avidities than for native FH. FH SCR17-20-binding anti-FH autoantibodies also bound the other cell-surface anchoring polyanionic binding site-containing region FH SCR5-8, at lower binding avidities. Anti-FH autoantibody avidities correlated with antibody titres. These anti-FH autoantibody characteristics did not differ between aHUS patients with or without the FHR1/3-/- genotype. Our data suggest a complex matrix of interactions between FHR1-FHR3 deletion, immunomodulation and anti-FH autoan...Continue Reading

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Citations

Apr 17, 2019·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Arvind BaggaUNKNOWN Indian Society of Pediatric Nephrology
Jan 2, 2021·Frontiers in Immunology·Yuzhou ZhangRichard J H Smith
Nov 3, 2020·Molecular Immunology·Felix Poppelaars, Joshua M Thurman

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