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Br J Haematol. 2017 Apr;177(1):136-141. doi: 10.1111/bjh.14502. Epub 2017 Feb 1.

Whole transcriptome sequencing identifies increased CXCR2 expression in PNH granulocytes.

Author information

1
Hematology Branch, National Heart, Lung, and Blood Institute (NHLBI), NIH, Bethesda, MD, USA.
2
BGI-Shenzhen, Shenzhen, China.
3
Trans-NIH Center for Human Immunology, Autoimmunity, and Inflammation, NIH, Bethesda, MD, USA.

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.

KEYWORDS:

CXCR2; RNA-sequencing; paroxysmal nocturnal haemoglobinuria

PMID:
28151558
PMCID:
PMC5378616
DOI:
10.1111/bjh.14502
[Indexed for MEDLINE]
Free PMC Article

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