Gurjar, B.S. and Manikanta, S.T. and Bhasym, A. and Prabhu, S. and Puraswani, M. and Khandelwal, P. and Saini, H. and Saini, S. and Verma, A.K. and Chatterjee, P. and Guchhait, P. and Bal, V. and George, A. and Rath, S. and Sahu, A. and Sharma, A. and Hari, P. and Sinha, A. and Bagga, A (2018) Characterization of geneticpredisposition and autoantibody profile in atypical haemolytic-uraemic syndrome. Immunology, 154 (4). pp. 663-672.

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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 autoantibodies in the aetiopathogenesis of aHUS.

Item Type:	Article
Depositing User:	Mr. Rameshwar Nema
Date Deposited:	12 Feb 2020 06:31
Last Modified:	12 Feb 2020 06:31
URI:	http://nccs.sciencecentral.in/id/eprint/615

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