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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-?B 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

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Whole transcriptome sequencing identifies increased CXCR2 expression in PNH granulocytes.

Hosokawa Kohei K   Kajigaya Sachiko S   Keyvanfar Keyvan K   Qiao Wangmin W   Xie Yanling Y   Biancotto Angelique A   Townsley Danielle M DM   Feng Xingmin X   Young Neal S NS  

British journal of haematology 20170201 1


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<sup>-</sup> compared to GPI-AP<sup>+</sup> granulocytes. Macrophage migration inhibitory factor, a CXCR2 agonis  ...[more]

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