Factor H interferes with the adhesion of sickle red cells to vascular endothelium: a novel disease-modulating molecule.

ABSTRACT: Sickle cell disease is an autosomal recessive genetic red cell disorder with a worldwide distribution. Growing evidence suggests a possible involvement of complement activation in the severity of clinical complications of sickle cell disease. In this study we found activation of the alternative complement pathway with microvascular deposition of C5b-9 on skin biopsies from patients with sickle cell disease. There was also deposition of C3b on sickle red cell membranes, which is promoted locally by the exposure of phosphatidylserine. In addition, we showed for the first time a peculiar "stop-and-go" motion of sickle cell red blood cells on tumor factor-α-activated vascular endothelial surfaces. Using the C3b/iC3b binding plasma protein factor Has an inhibitor of C3b cell-cell interactions,

SUBMITTER: Lombardi E 

PROVIDER: S-EPMC6518911 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature