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. 2019 May 1;10:853. doi: 10.3389/fimmu.2019.00853

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Copyright © 2019 Valoti, Alberti, Iatropoulos, Piras, Mele, Breno, Cremaschi, Bresin, Donadelli, Alizzi, Amoroso, Benigni, Remuzzi and Noris.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Anti-FHs in aHUS patients and healthy controls. 305 aHUS patients were analyzed for anti-FHs through ELISA: 254 were carriers of 1 or 2 copies of CFHR1 (aHUS with CFHR1), and 51 had 0 copies of CFHR1 (aHUS with HomCFHR1Del). Patient 23, carrying the heterozygous CFHR1Del and a frameshift variant in CFHR1 exon 2 (c.104delAfsX), which resulted in a complete deficiency of FHR1 has been also included in the group of patients defined as “aHUS with HomCFHR1Del.” Ninety-eight healthy controls with 1 or 2 copies of CFHR1 were also analyzed for anti-FHs (CTR). The positive threshold was set at mean +2SD of values recorded in the 98 controls (56 AU/mL) and is shown with the horizontal red dashed line.