Niemann-pick type C2 deficiency in human fibroblasts confers robust and selective activation of prostaglandin E2 biosynthesis

Abstract

Background: NPC2 is a protein that negatively regulates fibroblast activation. Results: NPC2-null human fibroblasts display induction of cPLA2, COX-2, and mPGES-1 expression that may contribute to the observed robust and selective activation of PGE2 biosynthesis. Conclusion: NPC2 may regulate the activated fibroblast inflammatory program through modulation of a cPLA2-dependent biosynthetic pathway. Significance: NPC2 may represent a novel therapeutic tool for the treatment of inflammatory diseases. © 2013 by The American Society for Biochemistry and Molecular Biology, Inc.