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PubMed1980

Immotile cilia syndrome in persons with and without Kartagener's syndrome.

We studied the ultrastructure and function of respiratory tract cilia in a group of patients with Kartagener's Syndrome, and compared the results with those from a group of subjects with unexplained histories of chronic bronchiectasis and bronchitis. Both patient groups lacked pulmonary and nasal mucociliary transport. On electron microscopic examination of nasal mucosal biopsy tissue, all patient specimens had abnormal cilia with a highly disorganized microtubular network, and a consistent absence of dynein arms. Cilia from both groups were immotile when viewed under direct phase contrast, but could be animated by the addition of 10(-6) g/ml of ATP or ATPase to the same degree of spontaneous motility seen in normal cilia. Immotile cilia syndrome clearly includes not only patients with Kartagener's syndrome, but also some patients with chronic bronchitis and bronchiectasis.

Adenosine Triphosphatases↗

PubMedDate not supplied

Scanning electron microscopic examination of nonbeating cilia.

When cilia stop beating, their role in moving mucus ceases, but it is uncertain if nonmotile cilia preserve their external architecture and are thus capable of maintaining an additional role of retarding microbial access to the cell. Cilia of chicken embryo tracheal organ cultures were observed until their activity stopped. When examined with scanning electron microscopy, ciliary axonemes did not appear to differ significantly from normally functioning cilia when observed at lower magnifications. Since their density can remain essentially unchanged, nonbeating cilia may still have a role in protecting respiratory membrane from toxic microorganisms.

Animals↗

PubMed2005

Cilia-driven fluid flow in the zebrafish pronephros, brain and Kupffer's vesicle is required for normal organogenesis.

Cilia, as motile and sensory organelles, have been implicated in normal development, as well as diseases including cystic kidney disease, hydrocephalus and situs inversus. In kidney epithelia, cilia are proposed to be non-motile sensory organelles, while in the mouse node, two cilia populations, motile and non-motile have been proposed to regulate situs. We show that cilia in the zebrafish larval kidney, the spinal cord and Kupffer's vesicle are motile, suggesting that fluid flow is a common feature of each of these organs. Disruption of cilia structure or motility resulted in pronephric cyst formation, hydrocephalus and left-right asymmetry defects. The data show that loss of fluid flow leads to fluid accumulation, which can account for organ distension pathologies in the kidney and brain. In Kupffer's vesicle, loss of flow is associated with loss of left-right patterning, indicating that the 'nodal flow' mechanism of generating situs is conserved in non-mammalian vertebrates.

Animals↗

PubMed1978

Transmission electron microscope observation of epithelial cells with single cilia in intrahepatic biliary ductules of bats.

Cells with long single cilia arising from basal bodies in the apical cytoplasm were occasionally revealed in the bile ductular epithelia of bats (Miniopterus schreibersi (Kuhl), Myotis macroductylus (Temminck) and Rhinolophus cornutus (Temminck)). The basal body (distal centriole) was associated witha proximal centriole, so the basal structure was of "two centriole type." In cross sections of the long tapering cilia the arrangement of cilary microtubules was determined. In the most proximal portion of the cilia doublet microtubules were arranged in the 9+0 pattern, while in more distal portions alteration and diminution of the doublet fibers occurred, splitting entirely into single microtubuli which were most frequently rearranged in the 6+1 or 7+1 pattern. The occurrence of the 9+0 fiber pattern and the basal structure of "two centriole type" suggested that the biliary ductular cilia might be sensory or chemoreceptive in nature and not motile. Similar cilia are expected to be found distributed widely in the epithelia of excretory ductal system of large exocrine glands of vertebrate species.

Animals↗

PubMed2003

Cilia ultrastructure in children with Down syndrome.

Chronic sinusitis, otitis media with effusion, and upper respiratory tract infections are commonly found in patients with Down syndrome. These diseases are generally felt to be secondary to depressed immune function and altered craniofacial dimensions. Recently, a cilia ultrastructure abnormality was found in a child with Down syndrome. This study is the first to be carried out to determine if cilia ultrastructure abnormalities are prevalent in the population with Down syndrome. Four of 10 patients had documented cilia abnormalities, but these were present in the background of normal cilia, suggesting that they were the result rather than the cause of chronic sinusitis. Similarly, nasal epithelium metaplasia was detected in 50% of the patients. Chronic sinusitis, otitis media with effusion, and recurrent upper respiratory tract infections in children with Down syndrome cannot generally be attributed to primary cilia ultrastructure abnormalities.

Adolescent↗

PubMed1988

Incidence and ultrastructure of rudimentary cilia in benign and malignant peripheral nerve tumors.

The incidence and ultrastructure of cilia were examined in 26 peripheral nerve tumors. The cilia had the common features of elongated shafts and basal structures consisting of two basal bodies. The basal body contained two basal feet and some long striated rootlets. The cilia lacked central tubules and two dynein arms of subfiber A, suggesting that they were nonmotile and rudimentary. The quantitative results showed that the incidence of cilia was low in Schwann cells and fibroblasts of the 20 benign tumors and slightly higher in perineurial cells of the 12 neurofibromas. Conversely, the incidence was high in most of the 6 malignant tumors. Only one perineurial cell from normal mature nerve examined carried a cilium, although no cilia were seen in the other perineurial cells, Schwann cells, and fibroblasts observed. The results showed that ciliary formation was more marked in the more actively proliferating tissues such as malignant tumors, and less so in the less actively proliferating tissues such as benign tumors. Ciliary formation may be one of the ultrastructural features commonly observed in proliferative tissues.

Cilia↗

PubMed1999

Asymmetry of cilia and of mice and men.

Evidence is given for the opinion that cilia in the early embryo, by their work, determine the laterality of the body; without ciliary work body laterality would be randomized. More exactly, monocilia in the primitive node are responsible for this determination. They have been described as being of the 9+0 type, but with dynein arms and with a gyrating movement. The orientation of the monocilia on the epithelium is of no importance but the direction of their gyration is, as may also be the shape of the node. The chirality of the cilia is thus reflected directly in the asymmetry of the body. The dynein arms go clockwise as seen from the base to tip and the ciliary rotation is in the same direction. The resulting waterflow is towards the left and so is the movement of the forming heart. In most subgroups of the immotile-cilia syndrome this mechanism does not work and equally many individuals will be born with situs inversus as with situs solitus. An exception is the immotile-cilia subgroup, named 'microtubule transposition', which is characterized by all cilia having a 9+0 structure throughout most of their length.

Animals↗

PubMed1989

Lack of cilia and squamous metaplasia in upper respiratory tract biopsies from children.

Biopsies were obtained from 47 children with upper respiratory tract infection aged 30 days - 12 years. All had been extensively investigated and no underlying cause for their symptoms could be found. A total of 63 specimens (46 from the inferior nasal turbinate, 8 from the trachea and 9 from the carina or bronchus) were examined. Only 4 showed no degree of squamous metaplasia and 8 showed significant numbers of acquired-type morphological abnormalities of the cilia. Twenty-six of the 63 biopsies had significantly reduced numbers of cilia and 34 had no cilia. Inherited-type changes were seen in only 1 patient. The net result of a lack of cilia and the immotile-cilia syndrome is the same--absence of mucociliary transport.

Biopsy↗

PubMedDate not supplied

[The absence of the internal arms of dynein as a cause of the immotile cilia syndrome].

Two children aged 9 and 8 years respectively presenting upper and lower respiratory tract infections from early childhood are presented. In both, nasal mucociliary transport was impaired (greater than 30 min) and electron microscopic examination showed cilia devoided of the inner dynein arms. This ciliary defect is one of those recognized as a cause of the Immotile Cilia Syndrome but is very rare, having been described only once in a boy affected with Kartagener's syndrome, which in turn has been reclassified in the Immotile Cilia Syndrome. Therefore the absence of inner dynein arms observed by us in the respiratory cilia of these two children clearly indicated that similarly to many other genetic disorders the immotile cilia syndrome may be determined by many defects in the axonemal structure.

Child↗

PubMed1983

[Presence of microcilia on the apical extremity of the cilia of the epithelium of the Fallopian tubes].

The role played by the ciliated cells of the salpinx in the transport of the ovum has been the subject of numerous studies. In this work we examined the ultrastructure of the cilia of the ampullary portion of the oviductal epithelium in a series of mammals and in man. We found the classical structure of the motile cilia. The free part of the cilia, surrounded by the cell membrane, is composed by nine peripheral doublets and by a pair of central microtubules. In the species we examined, we had the occasion to observe an ultrastructural particularity of the cilia. It is about a group of microcilia placed on their apical extremity. These formations were observed in longitudinal sections and appeared aligned on the top of the cilium. They have a length of 200-300 A and a diameter of 100 A. Their number varies with the species examined. The proportion of cilia which presented microcilia is also different from species to species. The functional role of the microcilia is unknown.

Animals↗

PubMed1983

Biochemical studies of the excitable membrane of Paramecium tetraurelia. VII. Sterols and other neutral lipids of cells and cilia.

The neutral lipid content of cells and cilia of Paramecium tetraurelia was determined as a function of growth stage and of growth medium composition. The major sterol(s) of deciliated cells and of cilia were the sterol provided in the growth medium (e.g., stigmasterol) and its 7-dehydro derivative. Body sterol esters and triglycerides accumulated during exponential cell growth and became depleted during stationary phase. Isolated cilia contained neither sterol esters nor triglycerides. The ratio of phospholipid to free sterol in cilia was constant (approximately 5:1) throughout exponential growth in axenic or bacterized medium, but the relative proportions of free sterol and 7-dehydrosterol varied with growth phase and with growth medium composition. Cholesterol did not support the growth of Paramecium, but was taken up rapidly into cells and cilia as the free sterol and its 7-dehydro derivative. The fatty acids of sterol esters and of triglycerides were similar to those of membrane phospholipids.

Animals↗

PubMed1995

Atypical cilia in the oviductal epithelium of healthy cats.

Specimens of the uterine tube were obtained from ten healthy, ovario-hysterectomized cats. Ultrastructurally, a total of 66,000 cilia were examined. Atypical cilia were found in all examined subjects. The prevalence of compound cilia showed a range of 0.2% to 1.8%. Intracytoplasmic and swollen cilia ranged from 0.06% to 0.3% and from 0.03% to 0.3%, respectively. The microtubular pattern was examined in 7,000 cross-sectioned cilia and an abnormal pattern was found in 2.6%-6.5%. Abnormalities of peripheral microtubules were the prevailing defect. A central plug of electron-dense material was observed in 2-3% of the examined basal bodies. Several basal bodies characterized by an abnormal spatial configuration were also shown.

Animals↗

PubMed1998

Ultrastructural and freeze fracture cilia morphology of trachea epithelium in apparently healthy small ruminants.

Specimens of tracheal mucosa were obtained from ten adult apparently healthy small ruminants (five goats and five sheep) both by a fibre optic endoscope and in a slaughterhouse. Ultrastructurally, a total of 50,000 cilia were examined. Pathological cilia were found in all examined subjects. The prevalence of compound cilia showed a range of 0.3% to 3%. Intracytoplasmic and swollen cilia ranged from 0.2% to 0.5%. The microtubular pattern was examined in 4,000 cross-sectioned cilia and an abnormal pattern was found in 5-7%. Microtubular defects involved both peripheral and central doublets, being peripheral abnormalities the prevailing ones. A central plug of electron dense material was observed in 2-3% of the examined basal bodies. Rare basal bodies characterized by an abnormal spatial configuration were also shown. Freeze fracture studies revealed a ciliary necklace composed of 4 to 5 rows of intramembrane particles.

Animals↗

PubMed1998

Atypical cilia in the oviductal epithelium of healthy dogs.

Specimens of the uterine tube (ampulla) were obtained from seven healthy, ovario-hysterectomized dogs. Ultrastructurally, a total of 35,000 cilia were examined. Compound cilia ranged from 0.0 to 0.4%; both intracytoplasmic and swollen cilia ranged from 0.1% to 0.4%. The microtubular pattern was studied in 3,500 cross-sectioned cilia and an abnormal pattern was found in 2-5%. Similarly to the other animal species, abnormalities involving the peripheral microtubules were the prevailing defect. An electron-dense plug into the lumen was seen in 1-3% of the basal bodies; occasionally an abnormal spatial configuration of them was also observed. The incidence of abnormal cilia hence is lower than found in the tracheae.

Animals↗

PubMed2000

Different frequency of cilia with transposition in human nasal and bronchial mucosa. A case of acquired ciliary dyskinesia.

Nasal and bronchial cilia and spermatozoa of a patient with a high clinical suspicion of a ciliary dyskinesia syndrome were ultrastructurally studied and quantified. Defective cilia showed two types of axonemal patterns: 9d+0s and 8d+1d. Of these, 9d+0s cilia prevailed in the proximal region, whereas 8d+1d prevailed in the distal region. Translocation of a peripheral doublet to the central position occurred at the middle region of cilia lacking the central pair, probably to compensate for its absence. Quantitative analysis showed that the percentages of anomalous cilia were 5.32+/-0.93 in nasal samples and 43.17+/-2.34 in bronchial samples. Spermatozoa without the central pair or with a translocated microtubular doublet were rarely observed, but a variety of nonspecific defects were seen. Even though transposition is generally considered to be an inherited ciliary defect and one of the causes of primary ciliary dyskinesia, in this case quantitative ultrastructural analysis and clinical data indicate that this is an acquired ciliary defect.

Adult↗

PubMed2004

Two anterograde intraflagellar transport motors cooperate to build sensory cilia on C. elegans neurons.

Cilia have diverse roles in motility and sensory reception and their dysfunction contributes to cilia-related diseases. Assembly and maintenance of cilia depends on the intraflagellar transport (IFT) of axoneme, membrane, matrix and signalling proteins to appropriate destinations within the organelle. In the current model, these diverse cargo proteins bind to multiple sites on macromolecular IFT particles, which are moved by a single anterograde IFT motor, kinesin-II, from the ciliary base to its distal tip, where cargo-unloading occurs. Here, we describe the observation of fluorescent IFT motors and IFT particles moving along distinct domains within sensory cilia of wild-type and IFT-motor-mutant Caenorhabditis elegans. We show that two anterograde IFT motor holoenzymes, kinesin-II and Osm-3-kinesin, cooperate in a surprising way to control two pathways of IFT that build distinct parts of cilia. Instead of each motor independently moving its own specific cargo to a distinct destination, the two motors function redundantly to transport IFT particles along doublet microtubules adjacent to the transition zone to form the axoneme middle segment. Next, Osm-3-kinesin alone transports IFT particles along the distal singlet microtubules to stabilize the distal segment. Thus, the subtle coordinate activity of these IFT motors creates two sequential transport pathways.

Animals↗

PubMed1997

Conjunctival cilia entrapment: an unrecognized cause of ocular irritation.

Conjunctival entrapment of cilia is form of ocular irritation that to our knowledge has not yet been described. Scarring predisposes the conjunctiva to this phenomenon by creating blind recesses in which loose cilia can become entrapped. Once caught, the rigid cilia can act as a mechanical irritant or act as a nidus for microbial growth. We present herein three patients with entrapped cilia and discuss their pathophysiology and management. The symptoms in all three patients resolved with conjunctivoplasty and removal of the cilia.

Adult↗

PubMed1986

Kartagener's syndrome. A blinded, controlled study of cilia ultrastructure.

We investigated respiratory mucosa cilia ultrastructure in patients homozygous for the gene for Kartagener's syndrome (KS) and patients apparently phenotypic for KS who had bronchiectasis and sinusitis but without situs inversus. Parents, as obligate carriers of the recessive KS gene, were also evaluated among other control groups. The four patients with KS had significantly fewer cilia outer dynein arms than normal subjects or parents of patients with KS. Two of five patients apparently phenotypic for KS demonstrated distinctive ultrastructural changes. No other subjects demonstrated explicit ultrastructural abnormalities. Internal control specimens showed that the number of outer dynein arms was consistent within a subject compared with variation between subjects. The outer dynein arm serves as a dependable ultrastructural marker. Carriers of KS do not demonstrate distinctive morphologic cilia abnormalities. Not every patient with chronic bronchiectasis and sinusitis demonstrates abnormal cilia ultrastructure.

Adolescent↗