Immotile cilia syndrome: Reduced chemotaxis and reduced number of intramembranous particles in granulocytes

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Abstract
The neutrophil granulocytes of four patients with immotile cilia syndrome were investigated by means of freeze-fracture technique. Whereas most granulocytic functions (adherence, phagocytosis, killing of micro-organisms, reduction of NBT, and chemoluminescence) were in the normal range, chemotaxis of the neutrophils was clearly reduced; their plasma membrane revealed a profound reduction in the density of intramembrane particles. An interrelationship between reduced particle density and defective chemotaxis in neutrophils is assumed, but not yet proven.