The Immotile-Cilia Syndrome: A Microtubule-Associated Defec
- 1 January 1985
- journal article
- review article
- Published by Informa UK Limited in Critical Reviews in Biochemistry
- Vol. 19 (1), 63-87
- https://doi.org/10.3109/10409238509086788
Abstract
The immotile-cilia syndrome is a congenital disorder characterized by all the cilia in the body being either immotile or showing an abnormal and inefficient beating pattern. Most symptoms come from the ciliated airways (nose, paranasal sinuses, and bronchs) and from the middle ear. Two further symptoms are situs inversus and male sterility. Situs inversus occurs in 50% of the cases and this subgroup is termed the Kartagener's syndrome; it might be due to an inability of the embryonic cilia to shift the heart to the left side and situs laterality seems to be a random process in the immotile-cilia syndrome. Male sterility is caused by the spermatozoa being unable to swim progressively; the sperm tail has the same structure as a cilium. In a few cases only the sperm tail or only the cilia of the body are affected. Female patients have a decreased fertility; most are involuntarily childless. The immotile-cilia syndrome is a heterogeneous disorder in that one out of many different genes may be involved. The different subtypes can be distinguished by an electron microscopic examination which will show defects in either one of a number of the ciliary components.Keywords
This publication has 116 references indexed in Scilit:
- Young's SyndromeNew England Journal of Medicine, 1984
- Kartagener's Syndrome with Motile SpermatozoaNew England Journal of Medicine, 1982
- The fine structure of the olfactory mucosa in manJournal of Neurocytology, 1982
- Polysplenia and Kartagener syndromes in a sibship: Association with abnormal respiratory ciliaThe Journal of Pediatrics, 1982
- Abnormal cilia in a child with the polysplenia syndrome and extrahepatic biliary atresiaThe Journal of Pediatrics, 1982
- Abnormal movement of polymorphonuclear neutrophils in the Immotile Cilia SyndromeExperimental Cell Research, 1981
- Ciliary motility in the ‘immotile cilia syndrome’: First results of microphoto-oscillographic studiesRespiratory Medicine, 1980
- Transposition of Ciliary MicrotubulesNew England Journal of Medicine, 1980
- Incidence and morphology of equine and murine chondrocytic ciliaThe Anatomical Record, 1980
- Structure and Function of Neutrophil Leukocytes from Patients with the Immotile‐Cilia SyndromeActa Medica Scandinavica, 1980