The role of properdin in complement-mediated renal diseases: a new player in complement-inhibiting therapy?

Pediatric Nephrology : Journal of the International Pediatric Nephrology Association
Marloes A H M MichelsLambertus P W J van den Heuvel

Abstract

Properdin is known as the only positive regulator of the complement system. Properdin promotes the activity of this defense system by stabilizing its key enzymatic complexes: the complement alternative pathway (AP) convertases. Besides, some studies have indicated a role for properdin as an initiator of complement activity. Though the AP is a powerful activation route of the complement system, it is also involved in a wide variety of autoimmune and inflammatory diseases, many of which affect the kidneys. The role of properdin in regulating complement in health and disease has not received as much appraisal as the many negative AP regulators, such as factor H. Historically, properdin deficiency has been strongly associated with an increased risk for meningococcal disease. Yet only recently had studies begun to link properdin to other complement-related diseases, including renal diseases. In the light of the upcoming complement-inhibiting therapies, it is interesting whether properdin can be a therapeutic target to attenuate AP-mediated injury. A full understanding of the basic concepts of properdin biology is therefore needed. Here, we first provide an overview of the function of properdin in health and disease. Then, we explore...Continue Reading

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Citations

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Oct 13, 2021·The Journal of Immunology : Official Journal of the American Association of Immunologists·Marloes A H M MichelsUNKNOWN COMBAT Consortium

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Methods Mentioned

BETA
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bronchoalveolar lavage
electron microscopy
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