Hemolytic uremic syndrome: How do factor H mutants mediate endothelial damage?

Trends in Immunology

Volumn 22, Issue 7, 2001, Pages 345-348

  Zipfel, Peter F ^a  

^a HANS KNÖLL INSTITUTE   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

COMPLEMENT FACTOR H; MUTANT PROTEIN; UREA;

CARBOXY TERMINAL SEQUENCE; COMPLEMENT ACTIVATION; DISEASE ACTIVITY; DISEASE ASSOCIATION; DISEASE COURSE; GENETIC ANALYSIS; HEMOLYTIC UREMIC SYNDROME; HUMAN; MUTATION; PATHOGENESIS; SHORT SURVEY; UREA BLOOD LEVEL;

EID: 0034920933     PISSN: 14714906     EISSN: None     Source Type: Journal    
DOI: 10.1016/S1471-4906(01)01972-X     Document Type: Review

References (21)

1. The hemolytic uremic syndrome
2. The molecular basis of familial hemolytic uremic syndrome: Mutation analysis of the factor H gene reveals a hot spot in short consensus repeat 20
3. Clustering of missense mutations in the C-terminal region of factor H in atypical hemolytic uremic syndrome
4. Factor H mutations in hemolytic uremic syndrome cluster in exons 18-20, a domain important for host-cell recognition
5. Each of the three binding sites on factor H interacts with a distinct site on C3b
6. Identification of three physically and functionally distinct binding sites for C3b in human complement factor H by deletion mutagenesis
7. Factor H and disease: A complement regulator affects vital body functions
8. The C-terminus of factor H: Monoclonal antibodies inhibit heparin binding and identify epitopes common to factor H and factor H-related proteins
9. Nephritogenic λ light-chain dimer: A unique human miniautoantibody against complement factor H
10. Human complement regulators: A major target for pathogenic microorganisms
11. Human factor H deficiency: Mutations in framework cysteine residues block factor H protein secretion and intracellular catabolism
12. Hereditary porcine membranoproliferative glomerulonephritis type II is caused by factor H deficiency
13. Complement factor H and hemolytic uremic syndrome
14. The complement system in hemolytic uremic syndrome in childhood
15. Hemolytic uremic syndrome in families
16. Hypocomplementemia discloses genetic predisposition to haemolytic uraemic syndrome and thrombotic thrombocytopenic purpura: Role of factor H abnormalities
17. Genetic studies into inherited and sporadic hemolytic uremic syndrome
18. Complement factor H gene mutation associated with autosomal recessive atypical hemolytic uremic syndrome
19. Complement activation occurs on subendothelial extracellular matrix in vitro and is initiated by retraction or removal of overlying endothelial cells
20. Interferon γinduces synthesis of complement alternative pathway proteins by human endothelial cells in culture
21. Regulation of the activity of platelet bound C3 convertase of the alternative pathway of complement by platelet factor H