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PubMed1975

Ultrastructural demonstration of cilia and ciliary rootlets in mammalian uterine tube epithelium in different functional states.

Tissue from the infundibulum region of the uterine tube (oviduct) of guinea pigs, cattle, sheep, and swine was examined by electron microscopy. In all specimens, cilia and ciliary rootlets were present in variable numbers in the ciliated cells during both the follicular and the luteal phases of the estrous cycle. True degeneration of cilia was not evident during luteal phase or pregnancy. The ciliary rootlets in ruminant and nonruminant species had structural similarities to those described in people and rhesus monkeys. These organelles measured approximately 1 mum long in most species studied, but in the cow, the rootlets, extending downward into the cytoplasm from the proximal end of the basal body, reached a length of 2 mum. The rootlets had a cross-striation of thick and thin bands, the period measuring 40 to 60 nm. Mitochondria were closely associated with the rootlets. The rootlets usually formed a small angle to the axis of the cilium. These organelles probably function as anchoring or stabilizing structures for the motile cilia. Results indicated that the rootlets are more widely distributed in mammalian uterine tube cilia than previously postulated. Polyribosomes, microfilaments, microtubules, and electron-opaque fibrous granules were frequently seen in the cytoplasm of the ciliated cells. The presence of fibrous granules in close association with the basal bodies indicate that these granules have a role in the development of cilia and rootlets. Cilia and precursor fibrous granules were also seen in porcine fetal uterine tube epithelial cells.

Animals↗
PubMed1976

[Ciliated cells of corneal endothelium. Functional and morphological aspects compared to cilia of other organs].

The presence of ciliated cells has been demonstrated by scanning and transmission electron microscopy in corneal endothelium. This study has been carried out on human cornea in normal and pathological cases and on rabbit's normal cornea. Another study has been lead on regeneration of endothelium after freeze destruction. The cilia cells described have a normal structure. The cilia has nine peripheral groups of tubules without any tubule in the center. The cilia seem to be more numerous in case of corneal disease and during the regeneration of corneal endothelium. The authors compare the presence of ciliated cells in different tissues. This suggest that the potency to form single cilia is present in almost every kind of tissue but there is no proof of their pathological importance. In the corneal endothelium, as in the kidney, cilia seem to have no function of fluid transportation but the possibility for cilia to be a receptor is discussed.

Animals↗
PubMed1989

Atypical cilia in the choroid plexus of guineapig.

Ultrastructural study of choroid plexus of normal and kaolin induced hydrocephalic guineapigs showed clusters of cilia arising from the apical part of some of the epithelial cells. Some of these cells had typical 9 + 2 cilia, while most of the control and the hydrocephalic groups showed 9 + 0 type of cilia. These atypical (9 + 0 type) cilia of choroidal epithelium differ from the primary cilia of other mammalian tissues in that, clusters of long cilia arise from a single cell and have no associated centrioles within the cytoplasm. They may be motile and sensory (osmoregulator or pressure sensor or both) in nature.

Animals↗
PubMed1986

The oviductal cilia and Kartagener's syndrome.

Women who have Kartagener's syndrome (primary ciliary dyskinesia) may or may not be fertile. The bronchial mucociliary clearance is reduced markedly in most of these women; this has led investigators to the conclusion that the cilia in the respiratory tract are immotile, and that "beating cilia may have no indispensable role in the female reproductive tract." Yet motile cilia are considered by many workers to be essential for normal ovum transport. More recently, bizarre ciliary motion has been described in the respiratory cilia of Kartagener's women. Our hypothesis was that the dyskinetic ciliary activity (or immotility) would be the same in both the respiratory and reproductive tracts and thus explain the fertility (or lack of it) in Kartagener's women. This report shows an identical ultrastructure and absolute immotility of cilia in both the respiratory tract and reproductive tract of a woman with Kartagener's syndrome who has never conceived. From this concordance, we suggest that the fertility of Kartagener's women is explained by the dyskinetic motion of oviductal cilia, and that the ciliated endosalpinx is essential for human reproduction.

Adult↗
PubMed1978

Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function.

Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of cilia. The results were compared with those of patients with congenitally immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all the seven CF patients who succeeded in inhaling the test aerosol. Ciliary ultrastructure from a deceased CF patient was normal. Patients with the immotile cilia syndrome had no substantial clearance and defective cilia. The CF patients coughed more during the clearance measurements than any other group studied earlier, and their coughing was effective. One patient succeeded in avoiding coughing in both measurements and had faster clearance when he got terbutaline than when he got the vehicle. Although younger, the CF patients tended to be more obstructed in their lungs and more handicapped than the patients suffering from the immotile-cilia syndrome. The latter patients had more discomfort from rhinitis, sinusitis and otitis than had the CF patients. An impairment of the mucociliary transport rate is hence unlikely to be a primary pathogenic factor for the respiratory tract disease in CF patients.

Adult↗
PubMed1983

Acquired ciliary defects compared to those seen in the immotile-cilia syndrome.

An attempt is made to characterize injured cilia and ciliary mutants separately with particular emphasis on human conditions. The usefulness of electron microscopical investigations is stressed. An injury to a ciliated epithelium may very well be restricted to one site in the body and may appear at any stage of life. Typical ciliary mutants, such as are seen in the immotile-cilia syndrome, represent a generalized condition and will be found from the beginning of life. Some types of ciliary defects are relatively unspecific and found after various kinds of insult to the cilia also in the immotile-cilia syndrome; these are: compound cilia, supernumerary microtubules in the axoneme, and loss of cilia or of ciliated cells. Other kinds of ciliary defect seem to be typical of the ciliary mutants: absence of dynein arms, absence of spokes and central sheath, transposition of one of the central microtubules and short central microtubules.

Anesthetics, Local↗
PubMed1981

The prevalence of abnormal cilia in normal pediatric lungs.

Abnormal respiratory cells have been reported in cases of chronic pediatric respiratory infection. In some cases, there is a specific defect of most cilia, but in others there is a variety of derangements of the architecture of only a fraction of the cilia. Histologically normal lungs from two children without known respiratory disease were examined and found to contain many abnormal cilia of the polymorphic type, which accounted for 3% to 5% of all cilia. Abnormalities included (1) excess cytoplasmic matrix, (2) additions, deletions, or aberrant arrangements of the 9 + 2 tubular pattern, (3) multiple partial or complete cilia within one membrane, and (4) occasional marked disorganization of all cilia in a cell. Polymorphic ciliary abnormalities may be common in healthy children, and should be cautiously interpreted as a cause of chronic respiratory infection.

Child↗
PubMed1980

The immotile cilia syndrome: phase contrast light microscopy, scanning and transmission electron microscopy.

In the immotile cilia syndrome, transmission electron microscopy of the cilia shows abnormalities in the arrangement of the central pairs of tubules and in the dynein arms of the peripheral tubules, or in the radial spokes, We studied four nonrelated children, 9/12, 5, 6, and 6 years old, with situs inversus and a history of chronic sinusitis and bronchitis (Kartagener's syndrome) and four children in the same age group and with the same history, but without situs inversus. Under the phase contrast microscope no motile cilia were seen in the four patients with Kartagener's syndrome and in two of the four other children. Transmission electron microscopy showed aberrations in the cilia (absence of dyneim arms, random orientation of central tubules) in the patients with Kartagener's syndrome. Scanning electron microscopy revealed differences in morphology and arrangement of cilia between patients and controls. In the patients much more mucus was present on the mucosal surface. Furthermore, the cilia were in a state of disorder, with a multidirectional orientation instead of the parallel orientation seen in controls.

Child↗
PubMed1994

Immotile cilia syndrome: nasal mucociliary function and nasal ciliary abnormalities.

We present 17 patients with a typical symptomatology of immotile cilia syndrome, seven of them with complete situs inversus. Firstly, a study of the nasal mucociliary transport was made by means of the radioisotopic technique with serum albumin-Tc99m. In all cases there was absence of transport. Secondly, we studied the ultrastructure of the nasal cilia. Defects in the dynein arms were frequently found (65%). In two cases (11%) there were no cilia; in two other cases the cilia were normal, and in another two cases alterations of the central pair of microtubules were seen. Ciliary complexes were detected in all cases. We conclude that in patients with chronic or recurrent infections of the airways without known cause we must initiate a study of the nasal mucociliary transport. If this is absent or decreased, study of ciliary ultrastructure should be carried out. If mucociliary transport is normal, immotile cilia syndrome is ruled out and ultrastructural study of the cilia is not required.

Adolescent↗
PubMed1994

Tubulin and tektin in sea urchin embryonic cilia: pathways of protein incorporation during turnover and regeneration.

Axonemal precursor tubulin is the major protein component of the detergent-soluble membrane/matrix fraction of sea urchin embryonic cilia. Its unusual abundance may reflect the rapid turnover of these cilia, a process that is further documented here. However, whether during induced regeneration or normal turnover and growth, most other newly synthesized axonemal proteins are not detectable in the membrane/matrix fraction, raising the question of how non-tubulin precursors transit the growing cilium to the distal tip where assembly is generally thought to occur. Three potential explanations were considered: (1) the assembly of these components is proximal; (2) their relative concentration is too low to detect; or (3) tubulin alone is conveyed via a membrane/matrix pathway while most other axonemal proteins are transported in association with the axoneme. Light microscope autoradiography of axonemes pulse-chase labeled with [3H]leucine showed relatively uniform labeling, with no evidence for proximal incorporation. Fully grown cilia and cilia at early stages of regeneration were isolated from labeled embryos, fractionated into membrane/matrix, axonemal tubulin and architectural remnant components, and their labeled protein compositions were compared. Heavily labeled axonemal proteins, most notably the integral microtubule doublet component tektin-A, were not detected in the membrane/matrix fraction of emerging cilia, even though nearly half of the total ciliary tubulin appeared in that fraction, arguing against membrane-associated or soluble matrix transit for the architectural proteins at low concentrations. However, after thermal fractionation of axonemes from growing cilia, labeled proteins characteristic of the architectural remnant dominated the solubilized microtubule fraction, supporting axoneme-associated transport of the non-tubulin proteins during growth, in contrast to a membrane/matrix pathway for tubulin.

Animals↗
PubMed1997

Calcium entry through cyclic nucleotide-gated channels in individual cilia of olfactory receptor cells: spatiotemporal dynamics.

Transient elevations of intracellular Ca2+ play an important role in regulating the sensitivity of olfactory transduction, but such elevations have not been demonstrated in the olfactory cilia, which are the site of primary odor transduction. To begin to understand Ca2+ signaling in olfactory cilia, we used high-resolution imaging techniques to study the Ca2+ transients that occur in salamander olfactory receptor neurons (ORNs) as a result of cyclic nucleotide-gated (CNG) channel activation. To visualize ciliary Ca2+ signals, we loaded ORNs with the Ca2+ indicator dye Fluo-3 AM and measured fluorescence with a laser scanning confocal microscope. Application of the phosphodiesterase inhibitor IBMX increased fluorescence in the cilia and other neuronal compartments; the ciliary signal occurred first and was more transient. This signal could be abolished by lowering external Ca2+ or by applying LY83583, a potent blocker of CNG channels, indicating that Ca2+ entry through CNG channels was the primary source of fluorescence increases. Direct activation of CNG channels with low levels of 8-Br-cGMP (1 microM) led to tonic Ca2+ signals that were restricted locally to the cilia and the dendritic knob. Elevated external K+, which depolarizes cell membranes, increased fluorescence signals in the cell body and dendrite but failed to increase ciliary Ca2+ fluorescence. The results demonstrate the existence and spatiotemporal properties of Ca2+ transients in individual olfactory cilia and implicate CNG channels as a major pathway for Ca2+ entry into ORN cilia during odor transduction.

1-Methyl-3-isobutylxanthine↗
PubMed1998

Imaging odor-induced calcium transients in single olfactory cilia: specificity of activation and role in transduction.

The possibility that odor stimuli trigger distinct Ca2+ elevations within the cilia of vertebrate olfactory receptor neurons (ORNs) is a widely proposed concept. However, because of the small size of the olfactory cilia, the existence and properties of such Ca2+ elevations and their role in odor transduction are still unknown. We investigate odor-induced Ca2+ changes in individual olfactory cilia from salamander using the Ca2+ indicator dye fluo-3 in combination with laser scanning confocal microscopy. Single brief applications of odor ligand produce highly localized Ca2+ elevations in individual cilia lasting for several seconds. These Ca2+ signals originate in the cilia and depend entirely on Ca2+ entry through ciliary cyclic nucleotide-gated ion channels. The odor specificity of the Ca2+ rises implies a receptor-operated mechanism underlying odor detection. Each of the cilia on a receptor neuron functions as an independent biochemical compartment that can detect odorants and produce a Ca2+ transient with remarkably uniform properties in terms of kinetics and odor specificity. The rate of recovery of the odor-induced Ca2+ transients matches recovery from a short-term form of odor adaptation. Application of the membrane-permeant intracellular Ca2+ chelator BAPTA AM eliminates this odor adaptation. The results indicate that an olfactory cilium serves as a basic functional unit at the input level of the olfactory system, controlling both the specificity and sensitivity of odor detection.

Adaptation, Physiological↗
PubMed2005

[The syndrome of dyskinetic cilia as the cause of chronic sinusitis].

UNLABELLED: The syndrome of dyskinetic cilia is the cause of chronic inflammatory processes of upper and lower airways. The research studies concerning cilia's structure were held during 50s. About 20 types of cilia's defects were characterized by now, classified as inborn defects. OBJECTIVES: The presentation of the case is because of both the rarity of incidence and diagnostical's problem and treatment. In this case, we observed who re-occurrence of chronic inflammation accompanied by having infections of lower respiratory tract it is necessary to expand diagnosis to find out the reasons of such disease. MATERIALS AND METHODS: A 7 years old girl was admitted to our Clinic. Since couple years she has been under assistance of Specialist Clinic. She was also hospitalized because of aggravation inflammatory state of lower airways. After computers tomography of sinuses she was qualified to surgical treatment. During operation a material to study of cilia immobility was taken. The electron microscope study confirm the diagnosis of the group of dyskinetic cilia. RESULTS: The follow-up examination was held 14 months after operation. During this time the girl did not take any antibiotic (before antibiotherapy was held couple times during the year). Clinical status proved. Better results were also on computer tomography. CONCLUSIONS: In this case, re-occurrence, inflammatory process both upper and lower respiratory tract are necessary to expand the diagnostics to find out the cause of illnesses. The methods of treatments the group of in-movement's cilia based on the symptomatic treatment, but the role of physiotherapy of respiratory system is stressed as the operation of removal of secretion's retention. There is an important role of regular laryngology care and surgical treatment allow partly to control the disease.

Child↗
PubMed1963

STUDIES ON CILIA. THE FIXATION OF THE METACHRONAL WAVE.

Upon excision into spring water, the lateral cilia of the gill of the freshwater mussel Elliptio complanatus (Solander) stop beating, but 0.04 M potassium ion can activate the gill so that these cilia again beat with metachronal rhythm. One per cent osmium tetroxide quickly pipetted onto a fully activated gill fixes the lateral cilia in a pattern that preserves the form and arrangement of the metachronal wave, and permits the cilia to be studied with the electron microscope in all stages of their beat cycle. Changes are seen in the fixed active preparation that are not present in the inactive control, i.e., in the packing of the cilia, the position of the axis of the ciliary cross-section, and the diameter of the ring of peripheral filaments. Analysis of these parameters may lead to new correlations between ciliary fine structure and function.

Animals↗
PubMed1992

Ultrastructure of connecting cilia in different forms of retinitis pigmentosa.

The connecting (sensory) cilium of rods and cones is the stalk that separates the outer segment, which contains visual pigment in stacks of membrane discs, from the inner segment, which contains cytoplasmic organelles involved in protein synthesis. There are conflicting reports on the occurrence of abnormal motile cilia in patients with retinitis pigmentosa (RP) and very few ultrastructural studies of photoreceptor connecting cilia in retinas from patients with RP. Defective connecting cilia could lead to the outer segment atrophy and degeneration that are characteristic of RP. The present study addresses the hypothesis that photoreceptor connecting cilia, as observed in cross section by electron microscopy, are defective in RP. We examined retinas from five patients with RP and four controls and found morphologic defects in the connecting cilia of one RP patient with type 2 Usher syndrome (86% abnormal, P less than .0001) but not in our sample of patients with X-linked (n = 2), simplex (n = 1), or autosomal dominant (n = 1) RP.

Adolescent↗
PubMed2004

NDP kinase moves into developing primary cilia.

Inmunofluorescence staining of murine NIH3T3 fibroblasts grown at high density shows that conventional nucleoside diphosphate (NDP) kinases A and B localize to a sensory organelle, the primary cilium. Similar results are obtained with Xenopus A6 kidney epithelial cells, suggesting that NDP kinases are a universal component of the primary cilium. The translocation of NDP kinase into primary cilia depends on size, taking place only when cilia reach a critical length of 5-6 microm. In mature cilia, NDP kinases are distributed along the ciliary shaft in a punctate pattern that is distinct from the continuous staining observed with acetylated alpha-tubulin, a ciliary marker and axonemal component. Isolation of a fraction enriched in primary cilia from A6 cells led to the finding that ciliary NDP kinase is enzymatically active, and is associated with the membrane and the matrix, but not the axoneme. In contrast, acetylated alpha-tubulin is found in the axoneme and, to a lesser extent, in the membrane. Based on the tightly regulated translocation process and the subciliary distribution pattern of NDP kinase, we propose that it plays a role in the elongation and maintenance of primary cilia by its ability to regenerate the GTP utilized by ciliary microtubule turnover and transmembrane signaling.

Animals↗
PubMed1982

Microtubule capping structures at the tips of tracheal cilia: evidence for their firm attachment during ciliary bend formation and the restriction of microtubule sliding.

The distal tips of the central pair and A-microtubules are capped in mammalian and avian tracheal cilia. The capping structures are similar to those found in protozoan cilia and flagella [Dentler, 1981], and consist of a central microtubule cap that links the central microtubules to the membrane or to the ciliary crown and A-microtubule plugs that insert into the lumen of each of the A-microtubules. In contrast to the protozoan capping structures, each of the A-microtubule plugs is bound to the central microtubule cap by distal filaments. The ends of the central and outer doublet microtubules are tightly bound to the cap in both intact and in demembranated and reactivated tracheal cilia. Analysis of the displacement of the microtubule tips in cilia fixed at various bend angles revealed that the displacements of A-microtubules are only partially in agreement with those predicted by the sliding filament model [Satir, 1968]. These results are discussed with respect to the regulation of microtubule sliding in capped cilia and the role of the microtubule capping structures in microtubule assembly.

Animals↗
PubMedDate not supplied

Occurrence of cilia in exfoliated ovarian adenocarcinoma cells.

Cilia identifiable by light microscopy occur extremely rarely in malignant tumor cells. This report documents the presence of cilia on exfoliated malignant tumor cells shed from an ovarian adenocarcinoma. Cilia-bearing tumor cells were observed in abdominal and pleural fluids. These cilia occurred at atypical locations. They were observed as unipolar, bipolar, lateral, and pericellular structures. Diagnostic features and implications of cilia in relation to malignant cells are discussed.

Aged↗