Abstract |
In paroxysmal nocturnal hemoglobinuria (PNH), hematopoietic cells lacking glycosylphosphatidylinositol ( GPI)-linked proteins on their surface (GPI(neg)) exist alongside normal (GPI+) cells. Analysis of natural killer (NK) cell subsets in 47 PNH patients revealed that the ratio of CD56(bright):CD56(dim) NK cells differed in the GPI+ and GPI(neg) populations, with GPI(neg)CD56(bright) NK cells significantly more abundant in peripheral blood than their normal GPI+ counterparts. Indeed, GPI+CD56(bright) NK cells were not detected in the peripheral blood of some patients, suggesting their trafficking to a niche unavailable to the GPI(neg)CD56(bright) NK cell population. Defective cellular trafficking in this disease was supported by findings showing differential chemokine receptor expression between GPI+ and GPI(neg) NK cells and impaired stromal cell-derived factor 1 (SDF-1)-induced chemotaxis of GPI(neg) NK cells. Our results indicate a role for GPI-linked proteins in NK cell subset homeostasis and suggest that differential chemokine responses might contribute to the balance of GPI+ and GPI(neg) populations in this disease.
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Authors | Yasser M El-Sherbiny, Richard J Kelly, Anita Hill, Gina M Doody, Peter Hillmen, Graham P Cook |
Journal | Blood
(Blood)
Vol. 122
Issue 11
Pg. 1887-90
(Sep 12 2013)
ISSN: 1528-0020 [Electronic] United States |
PMID | 23881915
(Publication Type: Journal Article)
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Chemical References |
- CD56 Antigen
- CXCL12 protein, human
- CXCR4 protein, human
- Chemokine CXCL12
- GPI-Linked Proteins
- Receptors, CXCR4
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Topics |
- CD56 Antigen
(immunology, metabolism)
- Cell Line
- Cell Movement
(drug effects, immunology)
- Cells, Cultured
- Chemokine CXCL12
(immunology, pharmacology)
- Chemotaxis
(drug effects, immunology)
- Flow Cytometry
- GPI-Linked Proteins
(immunology, metabolism)
- Hemoglobinuria, Paroxysmal
(blood, immunology, pathology)
- Homeostasis
(immunology)
- Humans
- Immunophenotyping
- Killer Cells, Natural
(drug effects, immunology, metabolism)
- Receptors, CXCR4
(immunology, metabolism)
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