Published online 11 February 2009
Haematologica, Vol 94, Issue 3, 409-413 doi:10.3324/haematol.13733
Copyright © 2009 by Ferrata Storti Foundation
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Brief Reports

Shwachman-Diamond syndrome neutrophils have altered chemoattractant-induced F-actin polymerization and polarization characteristics

Claudia Orelio1, Taco W. Kuijpers2

1 Sanquin Research and Landsteiner Laboratory Dept. Blood Cell Research Phagocyte Laboratory, Amsterdam;
2 Emma Children’s Hospital, University of Amsterdam Academic Medical Centre, Amsterdam, The Netherlands

Correspondence: Taco W. Kuijpers, Emma Children’s Hospital, University of Amsterdam, Academic Medical Centre Meibergdreef 9 1105 AZ, Amsterdam, The Netherlands. E-mail:t.w.kuijpers{at}amc.uva.nl

ABSTRACT

Shwachman-Diamond syndrome is a hereditary disorder characterized by pancreatic insufficiency and bone marrow failure. Most Shwachman-Diamond syndrome patients have mutations in the SBDS gene located at chromosome 7 and suffer from recurrent infections, due to neutropenia in combination with impaired neutrophil chemotaxis. Currently, the role of the actin cytoskeleton in Shwachman-Diamond syndrome neutrophils has not been investigated. Therefore, we performed immunofluorescence for SBDS and F-actin on human neutrophilic cells. Additionally, we examined in control neutrophils and cells from genetically defined Shwachman-Diamond syndrome patients F-actin polymerization and cytoskeletal polarization characteristics upon chemoattractant stimulation. These studies showed that SBDS and F-actin co-localize in neutrophilic cells and that F-actin polymerization and depolymerization characteristics are altered in Shwachman-Diamond syndrome neutrophils as compared to control neutrophils in response to both fMLP and C5a. Moreover, F-actin cytoskeletal polarization is delayed in Shwachman-Diamond syndrome neutrophils. Thus, Shwachman-Diamond syndrome neutrophils have aberrant chemoattractant-induced F-actin properties which might contribute to the impaired neutrophil chemotaxis.

Key words: SBDS gene, Shwachman-Diamond syndrome, actin cytoskeleton, fMLP.




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