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Basic Immunology and Pathology
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Factor H and Atypical Hemolytic Uremic Syndrome: Mutations in the C-Terminus Cause Structural Changes and Defective Recognition Functions

Mihály Józsi, Stefan Heinen, Andrea Hartmann, Clemens W. Ostrowicz, Steffi Hälbich, Heiko Richter, Anja Kunert, Christoph Licht, Rebecca E. Saunders, Stephen J. Perkins, Peter F. Zipfel and Christine Skerka
JASN January 2006, 17 (1) 170-177; DOI: https://doi.org/10.1681/ASN.2005080868
Mihály Józsi
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Stefan Heinen
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Andrea Hartmann
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Clemens W. Ostrowicz
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Steffi Hälbich
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Heiko Richter
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Anja Kunert
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Christoph Licht
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Rebecca E. Saunders
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Stephen J. Perkins
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Peter F. Zipfel
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Christine Skerka
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Article Information

vol. 17 no. 1 170-177
DOI 
https://doi.org/10.1681/ASN.2005080868
PubMed 
16338962

Published By 
American Society of Nephrology
Print ISSN 
1046-6673
Online ISSN 
1533-3450
History 
  • Received for publication August 19, 2005
  • Accepted for publication October 15, 2005
  • Published online December 27, 2005.

Article Versions

  • Earlier version (December 7, 2005 - 06:43).
  • You are viewing the most recent version of this article.
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© 2006 American Society of Nephrology

Author Information

  1. Mihály Józsi*,
  2. Stefan Heinen*,
  3. Andrea Hartmann*,
  4. Clemens W. Ostrowicz*,
  5. Steffi Hälbich*,
  6. Heiko Richter*,
  7. Anja Kunert*,
  8. Christoph Licht†,
  9. Rebecca E. Saunders‡,
  10. Stephen J. Perkins‡,
  11. Peter F. Zipfel*§ and
  12. Christine Skerka*
  1. *Department of Infection Biology, Leibniz Institute for Natural Product Research and Infection Biology, Hans Knoell Institute, Jena, Germany; †Children’s Hospital of the University of Cologne, Pediatric Nephrology, Cologne, Germany; ‡Department of Biochemistry and Molecular Biology, Royal Free and University College Medical School, University College London, London, United Kingdom; and §Frierich Schiller University, Jena, Germany
  1. Address correspondence to:
    Dr. Christine Skerka, Department of Infection Biology, Leibniz Institute for Natural Product Research, and Infection Biology, Beutenbergstrasse 11a, 07745 Jena, Germany. Phone: +49-3641-656848; Fax: +49-3641-656902; E-mail: christine.skerka{at}hki-jena.de

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Journal of the American Society of Nephrology: 17 (1)
Journal of the American Society of Nephrology
Vol. 17, Issue 1
January 2006
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Factor H and Atypical Hemolytic Uremic Syndrome: Mutations in the C-Terminus Cause Structural Changes and Defective Recognition Functions
Mihály Józsi, Stefan Heinen, Andrea Hartmann, Clemens W. Ostrowicz, Steffi Hälbich, Heiko Richter, Anja Kunert, Christoph Licht, Rebecca E. Saunders, Stephen J. Perkins, Peter F. Zipfel, Christine Skerka
JASN Jan 2006, 17 (1) 170-177; DOI: 10.1681/ASN.2005080868

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Factor H and Atypical Hemolytic Uremic Syndrome: Mutations in the C-Terminus Cause Structural Changes and Defective Recognition Functions
Mihály Józsi, Stefan Heinen, Andrea Hartmann, Clemens W. Ostrowicz, Steffi Hälbich, Heiko Richter, Anja Kunert, Christoph Licht, Rebecca E. Saunders, Stephen J. Perkins, Peter F. Zipfel, Christine Skerka
JASN Jan 2006, 17 (1) 170-177; DOI: 10.1681/ASN.2005080868
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  • Unraveling the Effect of a Potentiating Anti-Factor H Antibody on Atypical Hemolytic Uremic Syndrome-Associated Factor H Variants
  • Unravelling the effect of a potentiating anti-Factor H antibody on atypical hemolytic uremic syndrome associated factor H variants
  • Potentiation of complement regulator factor H protects human endothelial cells from complement attack in aHUS sera
  • Disease-linked mutations in factor H reveal pivotal role of cofactor activity in self-surface-selective regulation of complement activation
  • Complement Factor H-Related 5-Hybrid Proteins Anchor Properdin and Activate Complement at Self-Surfaces
  • Pharmacology of Heparin and Related Drugs
  • Complement Factor H Antibodies from Lung Cancer Patients Induce Complement-Dependent Lysis of Tumor Cells, Suggesting a Novel Immunotherapeutic Strategy
  • The Major Autoantibody Epitope on Factor H in Atypical Hemolytic Uremic Syndrome Is Structurally Different from Its Homologous Site in Factor H-related Protein 1, Supporting a Novel Model for Induction of Autoimmunity in This Disease
  • A 'silent', new polymorphism of factor H and apparent de novo atypical haemolytic uraemic syndrome after kidney transplantation
  • Identification of Factor H-like Protein 1 as the Predominant Complement Regulator in Bruch's Membrane: Implications for Age-Related Macular Degeneration
  • Factors determining penetrance in familial atypical haemolytic uraemic syndrome
  • Tissue-Specific Host Recognition by Complement Factor H Is Mediated by Differential Activities of Its Glycosaminoglycan-Binding Regions
  • Overall Neutralization of Complement Factor H by Autoantibodies in the Acute Phase of the Autoimmune Form of Atypical Hemolytic Uremic Syndrome
  • Disease-associated N-terminal Complement Factor H Mutations Perturb Cofactor and Decay-accelerating Activities
  • Binding of the Human Complement Regulators CFHR1 and Factor H by Streptococcal Collagen-like Protein 1 (Scl1) via Their Conserved C Termini Allows Control of the Complement Cascade at Multiple Levels
  • Relative Role of Genetic Complement Abnormalities in Sporadic and Familial aHUS and Their Impact on Clinical Phenotype
  • Complement Regulator Factor H Mediates a Two-step Uptake of Streptococcus pneumoniae by Human Cells
  • Hyperfunctional C3 convertase leads to complement deposition on endothelial cells and contributes to atypical hemolytic uremic syndrome
  • Factor H-related protein 1 (CFHR-1) inhibits complement C5 convertase activity and terminal complex formation
  • Smallpox Inhibitor of Complement Enzymes (SPICE): Dissecting Functional Sites and Abrogating Activity
  • Mutations of Factor H Impair Regulation of Surface-bound C3b by Three Mechanisms in Atypical Hemolytic Uremic Syndrome
  • Anti factor H autoantibodies block C-terminal recognition function of factor H in hemolytic uremic syndrome
  • Differential Impact of Complement Mutations on Clinical Characteristics in Atypical Hemolytic Uremic Syndrome
  • The Host Immune Regulator Factor H Interacts via Two Contact Sites with the PspC Protein of Streptococcus pneumoniae and Mediates Adhesion to Host Epithelial Cells
  • Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome
  • Hemolytic Uremic Syndrome: A Factor H Mutation (E1172Stop) Causes Defective Complement Control at the Surface of Endothelial Cells
  • Genetics of HUS: the impact of MCP, CFH, and IF mutations on clinical presentation, response to treatment, and outcome
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