Skip to main content
NCBI home page
The American Journal of Pathology logoLink to The American Journal of Pathology
. 1993 Aug;143(2):643–647.

Immotile cilia syndrome in pigs. A model for human disease.

F Roperto 1, P Galati 1, P Rossacco 1
PMCID: PMC1887015  PMID: 8342607

Abstract

This paper describes the ultrastructural alterations observed in the tracheal epithelium of six sibling swine suffering from porcine immotile cilia syndrome (PICS) compared with those in human immotile cilia syndrome (HICS). As in some human cases, the tracheal epithelium of these pigs was lined by cilia-lacking cells. A variety of dynein defects in other pigs suffering from PICS have been previously observed. The spectrum of defects affords evidence that the PICS is genetically heterogeneous. Available data suggests that there are many similarities between HICS and PICS. Therefore, it is proposed that PICS may prove to be a useful animal model for the human disease.

Full text

PDF
643

Images in this article

644Figure 1
on p.644
644Figure 2
on p.644
644Figure 3
on p.644
644Figure 4
on p.644
645Figure 5
on p.645
645Figure 6
on p.645

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Afzelius B. A. Genetical and ultrastructural aspects of the immotile-cilia syndrome. Am J Hum Genet. 1981 Nov;33(6):852–864. [PMC free article] [PubMed] [Google Scholar]
  2. Baskerville A. Ultrastructural studies of the normal pulmonary tissue of the pig. Res Vet Sci. 1970 Mar;11(2):150–155. [PubMed] [Google Scholar]
  3. Boisvieux-Ulrich E., Sandoz D. Determination of ciliary polarity precedes differentiation in the epithelial cells of quail oviduct. Biol Cell. 1991;72(1-2):3–14. doi: 10.1016/0248-4900(91)90072-u. [DOI] [PubMed] [Google Scholar]
  4. De Santi M. M., Magni A., Valletta E. A., Gardi C., Lungarella G. Hydrocephalus, bronchiectasis, and ciliary aplasia. Arch Dis Child. 1990 May;65(5):543–544. doi: 10.1136/adc.65.5.543. [DOI] [PMC free article] [PubMed] [Google Scholar]
  5. Edwards D. F., Kennedy J. R., Patton C. S., Toal R. L., Daniel G. B., Lothrop C. D. Familial immotile-cilia syndrome in English springer spaniel dogs. Am J Med Genet. 1989 Jul;33(3):290–298. doi: 10.1002/ajmg.1320330303. [DOI] [PubMed] [Google Scholar]
  6. Edwards D. F., Kennedy J. R., Toal R. L., Maddux J. M., Barnhill M. A., Daniel G. B. Kartagener's syndrome in a chow chow dog with normal ciliary ultrastructure. Vet Pathol. 1989 Jul;26(4):338–340. doi: 10.1177/030098588902600409. [DOI] [PubMed] [Google Scholar]
  7. Fonzi L., Lungarella G., Palatresi R. Lack of kinocilia in the nasal mucosa in the immotile-cilia syndrome. Eur J Respir Dis. 1982 Nov;63(6):558–563. [PubMed] [Google Scholar]
  8. Gaillard D. A., Lallement A. V., Petit A. F., Puchelle E. S. In vivo ciliogenesis in human fetal tracheal epithelium. Am J Anat. 1989 Aug;185(4):415–428. doi: 10.1002/aja.1001850405. [DOI] [PubMed] [Google Scholar]
  9. Gordon R. E., Kattan M. Absence of cilia and basal bodies with predominance of brush cells in the respiratory mucosa from a patient with immotile cilia syndrome. Ultrastruct Pathol. 1984;6(1):45–49. doi: 10.3109/01913128409016664. [DOI] [PubMed] [Google Scholar]
  10. Jahrsdoerfer R., Feldman P. S., Rubel E. W., Guerrant J. L., Eggleston P. A., Selden R. F. Otitis media and the immotile cilia syndrome. Laryngoscope. 1979 May;89(5 Pt 1):769–778. doi: 10.1288/00005537-197905000-00015. [DOI] [PubMed] [Google Scholar]
  11. Matwijiw I., Thliveris J. A., Faiman C. Aplasia of nasal cilia with situs inversus, azoospermia and normal sperm flagella: a unique variant of the immotile cilia syndrome. J Urol. 1987 Mar;137(3):522–524. doi: 10.1016/s0022-5347(17)44097-3. [DOI] [PubMed] [Google Scholar]
  12. Menco B. P., Farbman A. I. Genesis of cilia and microvilli of rat nasal epithelia during prenatal development. III. Respiratory epithelium surface, including a comparison with the surface of the olfactory epithelium. J Anat. 1987 Jun;152:145–160. [PMC free article] [PubMed] [Google Scholar]
  13. Morrison W. B., Wilsman N. J., Fox L. E., Farnum C. E. Primary ciliary dyskinesia in the dog. J Vet Intern Med. 1987 Apr-Jun;1(2):67–74. doi: 10.1111/j.1939-1676.1987.tb01989.x. [DOI] [PubMed] [Google Scholar]
  14. Richard S., Nezelof C., Pfister A., de Blic J., Scheinmann P., Paupe J. Congenital ciliary aplasia in two siblings. A primitive disregulation of ciliogenesis? Pathol Res Pract. 1989 Aug;185(2):181–183. doi: 10.1016/S0344-0338(89)80248-1. [DOI] [PubMed] [Google Scholar]
  15. Roperto F., Galati P., Maiolino P., Papparella S. Atypical basal bodies in the oviductal mucosa (ampulla) of gilts with primary ciliary dyskinesia (PCD). J Submicrosc Cytol Pathol. 1990 Oct;22(4):587–589. [PubMed] [Google Scholar]
  16. Roperto F., Galati P., Troncone A., Rossacco P., Campofreda M. Primary ciliary dyskinesia in pigs. J Submicrosc Cytol Pathol. 1991 Apr;23(2):233–236. [PubMed] [Google Scholar]
  17. Sorokin S. P. Reconstructions of centriole formation and ciliogenesis in mammalian lungs. J Cell Sci. 1968 Jun;3(2):207–230. doi: 10.1242/jcs.3.2.207. [DOI] [PubMed] [Google Scholar]
  18. Torikata C., Kijimoto C., Koto M. Ultrastructure of respiratory cilia of WIC-Hyd male rats. An animal model for human immotile cilia syndrome. Am J Pathol. 1991 Feb;138(2):341–347. [PMC free article] [PubMed] [Google Scholar]
  19. Welch M. J., Stiehm E. R., Dudley J. P. Isolated absence of nasal cilia: a case report. Ann Allergy. 1984 Jan;52(1):32–34. [PubMed] [Google Scholar]
  20. de Santi M. M., Gardi C., Barlocco G., Canciani M., Mastella G., Lungarella G. Cilia-lacking respiratory cells in ciliary aplasia. Biol Cell. 1988;64(1):67–70. doi: 10.1016/0248-4900(88)90094-9. [DOI] [PubMed] [Google Scholar]

Articles from The American Journal of Pathology are provided here courtesy of American Society for Investigative Pathology

RESOURCES