C3 deposition glomerulopathy due to a functional Factor H defect

Kidney International

Volumn 75, Issue 11, 2009, Pages 1230-1234

  Habbig, Sandra ^a   Mihatsch, Michael J ^b   Heinen, Stefan ^c   Beck, Bodo ^a   Emmel, Mathias ^a   Skerka, Christine ^c   Kirschfink, Michael ^d   Hoppe, Bernd ^a   Zipfel, Peter F ^c,e   Licht, Christoph ^f,g,h  

^a UNIVERSITY OF COLOGNE   (Germany)

^b UNIVERSITY HOSPITAL BASEL   (Switzerland)

^c HANS KNÖLL INSTITUTE   (Germany)

^d UNIVERSITY OF HEIDELBERG   (Germany)

^e FRIEDRICH SCHILLER UNIVERSITY JENA   (Germany)

^f Division of Pediatric Nephrology

^g UNIVERSITY OF TORONTO   (Canada)

^h HOSPITAL FOR SICK CHILDREN   (Canada)

Author keywords

[No Author keywords available]

Indexed keywords

ALTERNATIVE COMPLEMENT PATHWAY C3 C5 CONVERTASE; COMPLEMENT COMPONENT C3; COMPLEMENT FACTOR H; FRESH FROZEN PLASMA; LYSINE; NEPHRITIC FACTOR;

ARTICLE; C3 DEPOSITION GLOMERULOPATHY; CASE REPORT; CFH GENE; CHILD; CLINICAL EXAMINATION; COMPLEMENT ALTERNATIVE PATHWAY; COMPLEMENT DEPOSITION; DISEASE SEVERITY; ECHOGRAPHY; FEMALE; GENE; GENE DELETION; GLOMERULONEPHRITIS; HEMATURIA; HUMAN; KIDNEY BIOPSY; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; PRIORITY JOURNAL; PROTEINURIA; SCHOOL CHILD;

CHILD; COMPLEMENT C3; COMPLEMENT C3 NEPHRITIC FACTOR; COMPLEMENT FACTOR H; COMPLEMENT PATHWAY, ALTERNATIVE; FEMALE; GLOMERULONEPHRITIS, MEMBRANOUS; HEMATURIA; HUMANS; PLASMA; PROTEINURIA; SIBLINGS;

EID: 67349180237     PISSN: 00852538     EISSN: 15231755     Source Type: Journal    
DOI: 10.1038/ki.2008.354     Document Type: Article

References (15)

1. Servais A, Fremeaux-Bacchi V, Lequintrec M et al. Primary glomerulonephritis with isolated C3 deposits: a new entity which shares common genetic risk factors with haemolytic uraemic syndrome. J Med Genet 2007; 44: 193-199.
2. Licht C, Heinen S, Jozsi M et al. Deletion of Lys224 in regulatory domain 4 of Factor H reveals a novel pathomechanism for dense deposit disease (MPGN II). Kidney Int 2006; 70: 42-50.
3. Pickering MC, Cook HT. Translational mini-review series on complement factor H: renal diseases associated with complement factor H: novel insights from humans and animals. Clin Exp Immunol 2008; 151: 210-230.
4. Zipfel PF, Heinen S, Jozsi M et al. Complement and diseases: defective alternative pathway control results in kidney and eye diseases. Mol Immunol 2006; 43: 97-106.
5. Appel GB, Cook HT, Hageman G et al. Membranoproliferative glomerulonephritis type II (dense deposit disease): an update. J Am Soc Nephrol 2005; 16: 1392-1403.
6. Licht C, Schlotzer-Schrehardt U, Kirschfink M et al. MPGN II - genetically determined by defective complement regulation? Pediatr Nephrol 2007; 22: 2-9.
7. Jozsi M, Manuelian T, Heinen S et al. Attachment of the soluble complement regulator factor H to cell and tissue surfaces: relevance for pathology. Histol Histopathol 2004; 19: 251-258.
8. Pavenstadt H, Kriz W, Kretzler M. Cell biology of the glomerular podocyte. Physiol Rev 2003; 83: 253-307.
9. Daha MR, Fearon DT, Austen KF. C3 nephritic factor (C3NeF): stabilization of fluid phase and cell-bound alternative pathway convertase. J Immunol 1976; 116: 1-7.
10. Schwertz R, Rother U, Anders D et al. Complement analysis in children with idiopathic membranoproliferative glomerulonephritis: a long-term follow-up. Pediatr Allergy Immunol 2001; 12: 166-172.
11. Jokiranta TS, Solomon A, Pangburn MK et al. Nephritogenic lambda light chain dimer: a unique human miniautoantibody against complement factor H. J Immunol 1999; 163: 4590-4596.
12. Meri S, Koistinen V, Miettinen A et al. Activation of the alternative pathway of complement by monoclonal lambda light chains in membranoproliferative glomerulonephritis. J Exp Med 1992; 175: 939-950.
13. Abrera-Abeleda MA, Nishimura C, Smith JL et al. Variations in the complement regulatory genes factor H (CFH) and factor H related 5 (CFHR5) are associated with membranoproliferative glomerulonephritis type II (dense deposit disease). J Med Genet 2006; 43: 582-589.
14. Rennke HG. Secondary membranoproliferative glomerulonephritis. Kidney Int 1995; 47: 643-656.
15. Licht C, Weyersberg A, Heinen S et al. Successful plasma therapy for atypical hemolytic uremic syndrome caused by factor H deficiency owing to a novel mutation in the complement cofactor protein domain 15. Am J Kidney Dis 2005; 45: 415-421.