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We now report that Indian paediatric <jats:styled-content style=\"fixed-case\">aHUS<\/jats:styled-content> patients without anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibodies also showed modestly higher frequencies of the <jats:italic><jats:styled-content style=\"fixed-case\">FHR<\/jats:styled-content>1\/3<\/jats:italic><jats:sup><jats:italic>\u2013\/\u2013<\/jats:italic><\/jats:sup> genotype. Further, when we characterized epitope specificities and binding avidities of anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibodies in <jats:styled-content style=\"fixed-case\">aHUS<\/jats:styled-content> patients, most anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibodies were directed towards the <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> cell\u2010surface anchoring polyanionic binding site\u2010containing C\u2010terminal short conservative regions (<jats:styled-content style=\"fixed-case\">SCR<\/jats:styled-content>s) 17\u201320 with higher binding avidities than for native <jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content>. <jats:styled-content style=\"fixed-case\">FH SCR<\/jats:styled-content>17\u201320\u2010binding anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibodies also bound the other cell\u2010surface anchoring polyanionic binding site\u2010containing region <jats:styled-content style=\"fixed-case\">FH SCR<\/jats:styled-content>5\u20138, at lower binding avidities. Anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibody avidities correlated with antibody titres. These anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibody characteristics did not differ between <jats:styled-content style=\"fixed-case\">aHUS<\/jats:styled-content> patients with or without the <jats:italic><jats:styled-content style=\"fixed-case\">FHR<\/jats:styled-content>1\/3<\/jats:italic><jats:sup><jats:italic>\u2013\/\u2013<\/jats:italic><\/jats:sup> genotype. Our data suggest a complex matrix of interactions between <jats:italic><jats:styled-content style=\"fixed-case\">FHR<\/jats:styled-content>1<\/jats:italic>\u2010<jats:italic><jats:styled-content style=\"fixed-case\">FHR<\/jats:styled-content>3<\/jats:italic> deletion, immunomodulation and anti\u2010<jats:styled-content style=\"fixed-case\">FH<\/jats:styled-content> autoantibodies in the aetiopathogenesis of <jats:styled-content style=\"fixed-case\">aHUS<\/jats:styled-content>.<\/jats:p>","DOI":"10.1111\/imm.12916","type":"journal-article","created":{"date-parts":[[2018,2,27]],"date-time":"2018-02-27T11:57:18Z","timestamp":1519732638000},"page":"663-672","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":17,"title":["Characterization of genetic predisposition and autoantibody profile in atypical haemolytic\u2013uraemic syndrome"],"prefix":"10.1111","volume":"154","author":[{"given":"Bahadur Singh","family":"Gurjar","sequence":"first","affiliation":[{"name":"National Institute of Immunology New Delhi India"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tholu","family":"Manikanta Sriharsha","sequence":"additional","affiliation":[{"name":"National Centre for Cell Science S. 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