Terminal complement effectors in atypical hemolytic uremic syndrome: C5a, C5b-9, or a bit of both?
other · Level V
Where this comes from
- Record sourced from PubMed, PMID 31229026.
- Also identified by DOI 10.1016/j.kint.2019.02.038.
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Abstract
The role of the terminal complement pathway as the cause of atypical hemolytic uremic syndrome (aHUS) is widely recognized, but the relative contribution of the effectors C5a/C5aR1 and C5b-9 to disease pathogenesis has not been defined. Using FH<sup>R/R</sup> mice carrying a factor H mutation that causes cell surface complement alternative pathway dysregulation, Ueda documented that in FH<sup>R/R</sup> mice, C5b-9 causes renal thrombotic microangiopathy (TMA) whereas C5a/C5aR drives macrovascular thrombosis. This commentary addresses the implications and limitations of this study.
Medical subject headings
- Atypical Hemolytic Uremic Syndrome
- Thrombosis