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Blood, 1 February 2008, Vol. 111, No. 3, pp. 1512-1514. Prepublished online as a Blood First Edition Paper on November 15, 2007; DOI 10.1182/blood-2007-09-109876. This Article Full Text Full Text (PDF) Supplemental Table and Figure All Versions of this Article: blood-2007-09-109876v1 111/3/1512    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Józsi, M. Articles by Skerka, C. Search for Related Content PubMed PubMed Citation Articles by Józsi, M. Articles by Skerka, C. Related Collections Immunobiology Brief Reports Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  IMMUNOBIOLOGY Brief Report Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency Mihály Józsi1, Christoph Licht2, Stefanie Strobel1, Svante L. H. Zipfel1, Heiko Richter1, Stefan Heinen1, Peter F. Zipfel1,3, and Christine Skerka1 1 Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; 2 Division of Nephrology, Hospital for Sick Children, Toronto, ON; and 3 Friedrich Schiller University, Jena, Germany Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors. We previously described the deficiency of factor H–related proteins CFHR1 and CFHR3 as predisposing factor for aHUS. Here we identify in an extended cohort of 147 aHUS patients that 16 juvenile individuals (ie, 11%) who either lacked the CFHR1/CFHR3 completely (n = 14) or showed extremely low CFHR1/CFHR3 plasma levels (n = 2) are positive for factor H (CFH) autoantibodies. The binding epitopes of all 16 analyzed autoantibodies were localized to the C-terminal recognition region of factor H, which represents a hot spot for aHUS mutations. Thus we define a novel subgroup of aHUS, termed DEAP HUS (deficiency of CFHR proteins and CFH autoantibody positive) that is characterized by a combination of genetic and acquired factors. Screening for both factors is obviously relevant for HUS patients as reduction of CFH autoantibody levels represents a therapeutic option. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this? This article has been cited by other articles: A. Lionet, F. Provot, F. Glowacki, V. Fremeaux-Bacchi, and M. Hazzan A case of adult atypical haemolytic uraemic syndrome related to anti-factor H autoantibodies successfully treated by plasma exchange, corticosteroids and rituximab NDT Plus, December 1, 2009; 2(6): 458 - 460. [Abstract] [Full Text] [PDF] C. Licht, F. G. Pluthero, L. Li, H. Christensen, S. Habbig, B. Hoppe, D. F. Geary, P. F. Zipfel, and W. H. A. Kahr Platelet-associated complement factor H in healthy persons and patients with atypical HUS Blood, November 12, 2009; 114(20): 4538 - 4545. [Abstract] [Full Text] [PDF] C. Abarrategui-Garrido, R. Martinez-Barricarte, M. Lopez-Trascasa, S. Rodriguez de Cordoba, and P. Sanchez-Corral Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome Blood, November 5, 2009; 114(19): 4261 - 4271. [Abstract] [Full Text] [PDF] K. E. Schmid-Kubista, N. Tosakulwong, Y. Wu, E. Ryu, L. A. Hecker, K. H. Baratz, W. L. Brown, and A. O. 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