Disturbed sialic acid recognition on endothelial cells and platelets in complement attack causes atypical hemolytic uremic syndrome

Author:

Hyvärinen Satu1,Meri Seppo12,Jokiranta T. Sakari1

Affiliation:

1. Department of Bacteriology and Immunology, and Research Programs Unit, Immunobiology, University of Helsinki, Helsinki, Finland; and

2. Helsinki University Hospital Laboratory, Helsinki, Finland

Abstract

Key Points Sialic acids are critical for factor H–mediated complement regulation on endothelial cells, erythrocytes, and platelets. Impaired ability of factor H mutants to simultaneously bind sialic acid and C3b on cells explains their association with aHUS.

Publisher

American Society of Hematology

Subject

Cell Biology,Hematology,Immunology,Biochemistry

Reference43 articles.

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2. Atypical hemolytic uremic syndrome.;Kavanagh;Semin Nephrol,2013

3. Complement: a key system for immune surveillance and homeostasis.;Ricklin;Nat Immunol,2010

4. Atypical hemolytic uremic syndrome.;Loirat;Orphanet J Rare Dis,2011

5. The molecular basis of familial hemolytic uremic syndrome: mutation analysis of factor H gene reveals a hot spot in short consensus repeat 20.;Caprioli;J Am Soc Nephrol,2001

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