Summary
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.
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Abbreviations
- ICS:
-
immotile cilia syndrome
- IMP:
-
intramembrane particles
- KS:
-
Kartagener syndrome
References
Arge E (1960) Transposition of the viscera and sterility in men. Lancet I:412–414
Afzelius BA, Eliasson R (1979) Flagellar mutants in man: On the heterogeneity of the immotile cilia syndrome. J Ultrastruct Res 69:43–52
Afzelius BA, Ewetz L, Palmblad J, Udén A-M, Venizelos N (1980) Structure and function of neutrophil leucocytes from patients with the immotile cilia syndrome. Acta Med Scand 208:145–154
Antonelli M, Modesti A, de Angelis M, Marcolini P, Lucarelli N, Crifo S (1981) Immotile cilia syndrome: Radial spokes deficiency in a patient with Kartagener's triad. Acta Pediatr Scand 70:571–573
Baccetti B, Burrini AG, Maver A, Pallini V, Renieri T (1979) “9+0” immotile spermatozoa in an infertile man. Andrologia 11:437–443
Baccetti B, Burrini AG, Pallini V (1980) Spermatozoa and cilia lacking axoneme in an infertile man. Andrologia 12:525–532
Burridge K, Kelly T, Connell L (1982) Proteins involved in the attachment of actin to the plasma membrane. Phil Trans R Soc Lond 299:291–300
Caleb M, Lecks H, South MA, Norman ME (1977) Letter to the editor. New Engl J Med 297:1012–1013
Eliasson R, Mossberg B, Camner P, Afzelius BA (1977) The immotile cilia syndrome. A congenital ciliary abnormality as an etiologic factor in chronic airway infections and male sterility. New Engl J Med 297:1–6
Gallin JJ, Rosenthal AS (1974) The regulatory role of divalent cations in human granulocyte chemotaxis. Evidence for an association between calcium exchanges and microtubule assembly. J Cell Biol 62:594–609
Geiger B (1983) Membrane-cytoskeleton interaction. Biochem Biophys Acta 737:305–342
Geiger B, Tokuyasu KT, Dutton AH, Singer SJ (1980) Vinculin, an intracellular protein localized at specialized sites where microfilament bundles terminate at cell membranes. Proc Natl Acad Sci USA 77:4127–4131
Goldman AS, Schochet GG, Howell JT (1980) The discovery of defects in respiratory cilia in the immotile cilia syndrome. J Pediatr 96:244–247
Herson FS, Murphy S (1980) Normal ciliary ultrastructure in children with Kartagener's syndrome. Ann Otol Rhinol Laryngol 89:81–83
Jonsson MS, McCormick JR, Gillies CG, Gondos B (1982) Kartagener's syndrome with motile spermatozoa. New Engl J Med 307:1131–1133
Katz SM, Damjanov J, Carver J, Spitzer S, Mason D, Kauffman LA, Solnick P (1977) Kartagener's syndrome and abnormal cilia. Letter to the editor. New Engl J Med 297:1011–1012
Lungarella G, Fonzi L, Burrini AG (1982) Ultrastructural abnormalities in respiratory cilia and sperm tails in a patient with Kartagener's syndrome. Ultrastruct Pathol 3:319–323
Malech HL, Root RK, Gallin JJ (1977) Structural analysis of human neutrophil migration. Centriole, microtubule, and microfilament orientation and function during chemotaxis. J Cell Biol 75:666–693
McDowell EM, Barrett LA, Harris CC, Trump BF (1976) Abnormal cilia in human bronchial epithelium. Arch Pathol Lab Med 100:429–436
Pedersen H, Rebbe H (1975) Absence of arms in the axoneme of immobile human spermatozoa. Biol Reprod 12:541–544
Pedersen H, Mygind N (1976) Absence of axonemal arms in nasal mucosa cilia in Kartagener's syndrome. Nature 262:494–495
Rooklin AR, McGeady SJ, Mikaelian DO, Soriano RZ, Mansmann HC (1980) The immotile cilia syndrome: A cause of recurrent pulmonary disease in children. Pediatrics 66:526–531
Rossman CM, Forrest JB, Ruffin RE, Newhouse MT (1980) Immotile cilia syndrome in persons with and without Kartagener's syndrome. Am Rev Resp Dis 121:1011–1016
Rott H-D (1979) Kartagener's syndrome and the syndrome of immotile cilia. Hum Genet 46:249–261
Rydgren L, Simmingsköld G, Bandmann U, Norberg B (1976) The role of cytoplasmic microtubules in polymorphonuclear leucocyte chemotaxis. Evidence for the release hypothesis by means of time-lapse analysis of PMN movement relative to dot-like attractants. Exp Cell Res 99:207–220
Sturgess JM, Chao J, Wong J, Aspin N, Turner JAP (1979) Cilia with defective radial spokes. A cause of human respiratory disease. New Engl J Med 300:53–56
Yahara J, Edelman GM (1975) Electron-microscopic analysis of the modulation of lymphocyte receptor mobility. Exp Cell Res 91:125–142
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Supported by the Deutsche Forschungsgemeinschaft (Wo 215/6-4)
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Wolburg, H., Dopfer, R., Schieferstein, G. et al. Immotile cilia syndrome: Reduced chemotaxis and reduced number of intramembranous particles in granulocytes. Klin Wochenschr 62, 1044–1046 (1984). https://doi.org/10.1007/BF01711728
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DOI: https://doi.org/10.1007/BF01711728