Immotile cilia syndrome: reduced chemotaxis and reduced number of intramembranous particles in granulocytes.
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.
D000328:Adult; D002462:Cell Membrane / Q000648:ultrastructure; D002634:Chemotaxis, Leukocyte; D002648:Child; D002925:Ciliary Motility Disorders / Q000276:immunology*; D003594:Cytoplasmic Granules / Q000648:ultrastructure; D005260:Female; D005614:Freeze Fracturing; D006098:Granulocytes / Q000276:immunology / Q000648:ultrastructure; D006801:Humans; D007619:Kartagener Syndrome / Q000276:immunology; D008297:Male; D009504:Neutrophils / Q000276:immunology / Q000648:ultrastructure*