Whole transcriptome sequencing identifies increased CXCR2 expression in PNH granulocytes.

ABSTRACT: The aetiology of paroxysmal nocturnal haemoglobinuria (PNH) is a somatic mutation in the X-linked phosphatidylinositol glycan class A gene (PIGA), resulting in global deficiency of glycosyl phosphatidylinositol-anchored proteins (GPI-APs). This study applied RNA-sequencing to examine functional effects of the PIGA mutation in human granulocytes. CXCR2 expression was increased in GPI-AP^- compared to GPI-AP⁺ granulocytes. Macrophage migration inhibitory factor, a CXCR2 agonist, was significantly higher in plasma of PNH patients. Nuclear factor-κB phosphorylation was upregulated in GPI-AP^- compared with GPI-AP⁺ granulocytes. Our data suggest novel mechanisms in PNH, not obviously predicted by decreased production of the GPI moiety.

SUBMITTER: Hosokawa K 

PROVIDER: S-EPMC5378616 | biostudies-literature | 2017 Apr

REPOSITORIES: biostudies-literature