Anti-factor H antibody affecting factor H cofactor activity in a patient with dense deposit disease

Clinical Kidney Journal
Pilar NozalMargarita Lopez-Trascasa

Abstract

Complement alternative pathway dysregulation seems to be the pathophysiological basis of Dense Deposit Disease (DDD). Here, we describe a monoclonal anti-factor H (FH) autoantibody in a woman diagnosed with DDD with a monoclonal gammapathy. Enzyme-linked immunosorbent assays evidenced the presence of anti-FH antibodies in the patient's serum and showed that they were associated with the monoclonal IgG-λ fraction. These autoantibodies recognize the N-terminal region of FH and interfere with its regulatory function. In summary, in the DDD patient described here, the activation of complement alternative pathway was favoured by the presence of anti-FH autoantibodies that recognize the regulatory region of this protein and impede its function and which could ultimately cause the glomerulopathy.

References

Jul 20, 2005·Molecular Immunology·Peter F ZipfelChristine Skerka
Apr 11, 2007·Current Opinion in Nephrology and Hypertension·Patrick D Walker
Aug 7, 2007·Journal of the American Society of Nephrology : JASN·Richard J H SmithUNKNOWN Dense Deposit Disease Focus Group
Feb 5, 2009·Thrombosis and Haemostasis·Christoph Licht, Veronique Fremeaux-Bacchi
Mar 3, 2010·Molecular Immunology·Stefanie StrobelMihály Józsi
Sep 14, 2010·American Journal of Kidney Diseases : the Official Journal of the National Kidney Foundation·Sanjeev SethiRichard J H Smith

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Citations

Jan 13, 2018·Frontiers in Immunology·Marcell CserhalmiMihály Józsi
Feb 27, 2018·BMJ Case Reports·Jose Luis Espinosa-FigueroaMercedes Velo-Plaza
Oct 4, 2017·Annals of Rehabilitation Medicine·Asayeon ChoiJoo Hyun Park
Jan 18, 2020·International Journal of Molecular Sciences·Francesco Paolo SchenaMichele Rossini
Jun 28, 2018·Pediatric Nephrology : Journal of the International Pediatric Nephrology Association·Marina NorisGiuseppe Remuzzi
Apr 29, 2015·The Journal of Immunology : Official Journal of the American Association of Immunologists·Caroline BlancMarie-Agnès Dragon-Durey
Jul 28, 2016·Nature Reviews. Nephrology·Marie-Agnes Dragon DureyArvind Bagga
Jun 19, 2013·The Journal of Immunology : Official Journal of the American Association of Immunologists·Mario HebeckerMihály Józsi
Dec 13, 2016·Cellular and Molecular Life Sciences : CMLS·Raffaella ParenteAnthony J Day
Feb 5, 2021·Clinical Journal of the American Society of Nephrology : CJASN·Mathieu LemaireVéronique Frémeaux-Bacchi

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Methods Mentioned

BETA
biopsy
ELISA
Enzyme-linked immunosorbent assay
electrophoresis

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