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Clinical and Experimental Medicine
Published in: Clinical and Experimental Medicine 3/2018

Open Access 01-08-2018 | Review Article

The role of the alternative pathway of complement activation in glomerular diseases

Authors: Emilia Łukawska, Magdalena Polcyn-Adamczak, Zofia I. Niemir

Published in: Clinical and Experimental Medicine | Issue 3/2018

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Abstract

The complement system (CS) has recently been recognized as a bridge between innate and adaptive immunity that constitutes a very complex mechanism controlling the clearance of pathogens, cellular debris, and immune complexes. Out of three known pathways of complement activation, the alternative pathway (AP) plays a critical role in host defense by amplifying the complement response, independently of initiation pathway and continuously maintaining low-level activity in a process called ‘thick-over.’ A key molecule of the CS is C3, in which the AP is constantly activated. To prevent host cell destruction, a group of the AP regulators tightly controls this pathway of the CS activation. Acquired and genetic abnormalities of the CS may alter the delicate balance between enhancing and inhibiting the AP cascade. These can lead to the uncontrolled CS activation, inflammatory response, and subsequent tissue damage. Since complement components are locally produced and activated in the kidney, the abnormalities targeting the AP may cause glomerular injury. C3 glomerulopathy is a new entity, in which the AP dysregulation has been well established. However, recent studies indicate that the AP may also contribute to a wide range of kidney pathologies, including immune-complex-mediated glomerulonephritis (GN), pauci-immune GN, and primary membranous nephropathy (PMN). This article provides insight into current knowledge on the role of the AP in the pathogenesis of glomerular diseases, focusing mainly on various types of primary and secondary GN and PMN.
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Metadata
Title
The role of the alternative pathway of complement activation in glomerular diseases
Authors
Emilia Łukawska
Magdalena Polcyn-Adamczak
Zofia I. Niemir
Publication date
01-08-2018
Publisher
Springer International Publishing
Published in
Clinical and Experimental Medicine / Issue 3/2018
Print ISSN: 1591-8890
Electronic ISSN: 1591-9528
DOI
https://doi.org/10.1007/s10238-018-0491-8

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