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 FHR/R mice carrying a factor H mutation that causes cell surface complement alternative pathway dysregulation, Ueda documented that in FHR/R mice, C5b-9 causes renal thrombotic microangiopathy (TMA) whereas C5a/C5aR drives macrovascular thrombosis. This commentary addresses the implications and limitations of this study.
|
Authors | Marina Noris, Giuseppe Remuzzi |
Journal | Kidney international
(Kidney Int)
Vol. 96
Issue 1
Pg. 13-15
(07 2019)
ISSN: 1523-1755 [Electronic] United States |
PMID | 31229026
(Publication Type: Journal Article, Comment)
|
Copyright | Copyright © 2019 International Society of Nephrology. Published by Elsevier Inc. All rights reserved. |
Chemical References |
- Complement Membrane Attack Complex
- Complement Factor H
|
Topics |
- Animals
- Atypical Hemolytic Uremic Syndrome
- Complement Factor H
(genetics)
- Complement Membrane Attack Complex
- Mice
- Mutation
- Thrombosis
|