Atypical hemolytic uremic syndrome associated with a factor B genetic variant and fluid-phase complement activation: an exception to the rule?

Abstract:

:Gain-of-function variants in CFB encoding factor B (FB), a component of the alternative pathway C3 convertase, have been reported in a minority of patients with aHUS and result in massive C3 activation. Zhang et al. describe the functional characterization of a novel FB variant (p.Ser367Arg) that they identified in 2 unrelated aHUS pedigrees who had undetectable C3 levels. The mutant FB caused strong C3 cleavage in fluid-phase but also C3 deposition on cell surface. This commentary addresses the implications of these findings for understanding the complexity of complement-related genetic renal diseases.

journal_name

Kidney Int

journal_title

Kidney international

authors

Noris M,Remuzzi G

doi

10.1016/j.kint.2020.06.026

subject

Has Abstract

pub_date

2020-11-01 00:00:00

pages

1084-1087

issue

5

eissn

0085-2538

issn

1523-1755

pii

S0085-2538(20)30814-0

journal_volume

98

pub_type

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