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10.1111/cei.12754

http://scihub22266oqcxt.onion/10.1111/cei.12754
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C4778104!4778104!26660535
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suck abstract from ncbi


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pmid26660535      Clin+Exp+Immunol 2016 ; 184 (1): 118-25
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  • Serum properdin consumption as a biomarker of C5 convertase dysregulation in C3 glomerulopathy #MMPMID26660535
  • Corvillo F; Bravo García?Morato M; Nozal P; Garrido S; Tortajada A; Rodríguez de Córdoba S; López?Trascasa M
  • Clin Exp Immunol 2016[Apr]; 184 (1): 118-25 PMID26660535show ga
  • Properdin (P) stabilizes the alternative pathway (AP) convertases, being the only known positive regulator of the complement system. In addition, P is a pattern recognition molecule able to initiate directly the AP on non?self surfaces. Although P deficiencies have long been known to be associated with Neisseria infections and P is often found deposited at sites of AP activation and tissue injury, the potential role of P in the pathogenesis of complement dysregulation?associated disorders has not been studied extensively. Serum P levels were measured in 49 patients with histological and clinical evidence of C3 glomerulopathy (C3G). Patients were divided into two groups according to the presence or absence of C3 nephritic factor (C3NeF), an autoantibody that stabilizes the AP C3 convertase. The presence of this autoantibody results in a significant reduction in circulating C3 (P < 0·001) and C5 levels (P < 0·05), but does not alter factor B, P and sC5b?9 levels. Interestingly, in our cohort, serum P levels were low in 17 of the 32 C3NeF?negative patients. This group exhibited significant reduction of C3 (P?
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