Literature DB >> 20118898

Excessive activation of the complement system in atypical hemolytic uremic syndrome: is it ready for prime time?

Han-Mou Tsai1.   

Abstract

Complement factor I (CFI) mutations are implicated in the pathogenesis of atypical hemolytic uremic syndrome (aHUS). Nevertheless, there is evidence that CFI deficiency is a weak effector of aHUS. Bienaime et al. report that homozygous deletion of CFHR-1 in the RCA gene cluster of chromosome 1q is a major risk factor for poor outcome for patients with CFI mutations. The basic and clinical implications of the findings are further elaborated here.

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Year:  2010        PMID： 20118898      PMCID： PMC3153079          DOI： 10.1038/ki.2009.467

Source DB:  PubMed          Journal:  Kidney Int        ISSN： 0085-2538            Impact factor:   10.612


  13 in total

1.  Eculizumab for atypical hemolytic-uremic syndrome.

Authors:  Jens Nürnberger; Thomas Philipp; Oliver Witzke; Anabelle Opazo Saez; Udo Vester; Hideo Andreas Baba; Andreas Kribben; Lothar Bernd Zimmerhackl; Andreas R Janecke; Mato Nagel; Michael Kirschfink
Journal:  N Engl J Med       Date:  2009-01-29       Impact factor: 91.245

Review 2.  Complement regulators and inhibitory proteins.

Authors:  Peter F Zipfel; Christine Skerka
Journal:  Nat Rev Immunol       Date:  2009-09-04       Impact factor: 53.106

3.  Deficiency of C3 inactivator in man.

Authors:  N Abramson; C A Alper; P J Lachmann; F S Rosen; J H Jandl
Journal:  J Immunol       Date:  1971-07       Impact factor: 5.422

4.  Mutations in components of complement influence the outcome of Factor I-associated atypical hemolytic uremic syndrome.

Authors:  Frank Bienaime; Marie-Agnes Dragon-Durey; Catherine H Regnier; Sara C Nilsson; Wing H Kwan; Jacques Blouin; Mathieu Jablonski; Nicolas Renault; Marie-Anne Rameix-Welti; Chantal Loirat; Catherine Sautés-Fridman; Bruno O Villoutreix; Anna M Blom; Veronique Fremeaux-Bacchi
Journal:  Kidney Int       Date:  2009-12-16       Impact factor: 10.612

Review 5.  Mechanisms of microvascular thrombosis in thrombotic thrombocytopenic purpura.

Authors:  Han-Mou Tsai
Journal:  Kidney Int Suppl       Date:  2009-02       Impact factor: 10.545

6.  Complement factor I: a susceptibility gene for atypical haemolytic uraemic syndrome.

Authors:  V Fremeaux-Bacchi; M-A Dragon-Durey; J Blouin; C Vigneau; D Kuypers; B Boudailliez; C Loirat; E Rondeau; W H Fridman
Journal:  J Med Genet       Date:  2004-06       Impact factor: 6.318

7.  Deletion of CFHR3 and CFHR1 genes in age-related macular degeneration.

Authors:  Kylee L Spencer; Michael A Hauser; Lana M Olson; Silke Schmidt; William K Scott; Paul Gallins; Anita Agarwal; Eric A Postel; Margaret A Pericak-Vance; Jonathan L Haines
Journal:  Hum Mol Genet       Date:  2007-12-15       Impact factor: 6.150

8.  Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation.

Authors:  Stefan Heinen; Andrea Hartmann; Nadine Lauer; Ulrike Wiehl; Hans-Martin Dahse; Sylvia Schirmer; Katharina Gropp; Tina Enghardt; Reinhard Wallich; Steffi Hälbich; Michael Mihlan; Ursula Schlötzer-Schrehardt; Peter F Zipfel; Christine Skerka
Journal:  Blood       Date:  2009-06-15       Impact factor: 22.113

9.  Factor I is required for the development of membranoproliferative glomerulonephritis in factor H-deficient mice.

Authors:  Kirsten L Rose; Danielle Paixao-Cavalcante; Jennifer Fish; Anthony P Manderson; Talat H Malik; Anne E Bygrave; Tao Lin; Steven H Sacks; Mark J Walport; H Terence Cook; Marina Botto; Matthew C Pickering
Journal:  J Clin Invest       Date:  2008-02       Impact factor: 14.808

10.  Thrombomodulin mutations in atypical hemolytic-uremic syndrome.

Authors:  Mieke Delvaeye; Marina Noris; Astrid De Vriese; Charles T Esmon; Naomi L Esmon; Gary Ferrell; Jurgen Del-Favero; Stephane Plaisance; Bart Claes; Diether Lambrechts; Carla Zoja; Giuseppe Remuzzi; Edward M Conway
Journal:  N Engl J Med       Date:  2009-07-23       Impact factor: 91.245
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