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Loss of properdin exacerbates C3 glomerulopathy resulting from factor H deficiency

Marieta M Ruseva1, Katherine A Vernon, Allison M Lesher

  • 1Centre for Complement and Inflammation Research, Imperial College, London, United Kingdom.

Journal of the American Society of Nephrology : JASN
|November 28, 2012
PubMed

Insights

Properdin influences complement C3 localization in the kidneys during uncontrolled complement activation caused by Complement factor H deficiency. Inhibiting properdin may worsen kidney disease in this context.

Area of Science:

  • Immunology
  • Nephrology
  • Complement System Biology

Background:

  • Complement factor H (CFH) normally inhibits the alternative complement pathway.
  • CFH deficiency in mice leads to uncontrolled C3 activation and kidney disease.
  • The role of properdin, the sole positive regulator of the alternative pathway, in CFH-deficiency-driven kidney disease is unknown.

Purpose of the Study:

  • To investigate the role of properdin in the pathophysiology of kidney disease in CFH-deficient mice.
  • To examine the effects of combined CFH and properdin deficiency on complement activation and C3 deposition in the kidney.

Main Methods:

  • Studied mice deficient in both CFH and properdin (CFH(-/-).P(-/-)).
  • Analyzed plasma complement profiles (C3, C5 levels).
  • Assessed glomerular inflammation, capillary wall thickening, and glomerular C3 staining.

Main Results:

  • CFH(-/-).P(-/-) mice showed predominant C3 depletion, unlike CFH(-/-) mice with C3 and C5 depletion.
  • Glomerular inflammation, capillary wall thickening, and C3 deposition were significantly increased in CFH(-/-).P(-/-) mice compared to CFH(-/-) mice.
  • The beneficial effects of exogenous CFH on glomerular C3 deposition in CFH(-/-) mice were dependent on properdin.

Conclusions:

  • Properdin influences the intraglomerular localization of C3 during uncontrolled C3 activation driven by CFH deficiency.
  • Therapeutic inhibition of properdin could be detrimental in settings of complete CFH deficiency and uncontrolled C3 activation.
  • Findings suggest a complex interplay between CFH and properdin in regulating complement-mediated kidney injury.

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