DGKE disruption ditches complement and drives p38 signaling

Abstract

In this issue of Blood, Bruneau et al1 provide evidence that disruption of diacylglycerol kinase e isoform (DGKe) does not upregulate complement activation, but rather induces endothelial damage via the activation of p38 mitogen-activated protein kinase (MAPK). These in vitro findings may support a new pathophysiologic mechanism for atypical hemolytic uremic syndrome (aHUS) in a subset of patients with DGKE gene mutations.