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At least 37 records · Page 2Linked to original sources
PubMed1997

Cilia from a cystic fibrosis patient react to the ciliotoxic Pseudomonas aeruginosa II lectin in a similar manner to normal control cilia--a case report.

The ciliary beat frequency measurements taken from a nasal polyp from a cystic fibrosis patient were similar to that of the control nasal polyps. The addition of a ciliotoxic lectin produced by Pseudomonas aeruginosa stopped the beating of the cilia as in the controls. This reaction could be blocked by the pre-incubation of the lectin with its inhibitor fucose. As in the control, the addition of fucose after the cilia had slowed resulted in a return to normal ciliary beating within 24 hours. This shows that the delta F508 CF mutation observed in this patient does not affect ciliary beating and suggests that treatment with fucose in the early stages of a Pseudomonas aeruginosa infection could be advantageous for cystic fibrosis patients.

Adhesins, Bacterial↗
PubMed1979

Activation of nasal cilia in immotile cilia syndrome.

Nasal biopsy specimens were obtained from 5 normal subjects and from 7 patients with immotile cilia syndrome. Of the latter, 3 had Kartagener's syndrome, one had Kartagener's forme fruste, and 3 had bronchiectasis and sinusitis. An in vitro motility test was used to assess ciliary movement. Exogenous adenosine triphosphate and adenosine triphosphatase activated the immotile cilia to levels equal to or slightly greater than the spontaneous activity seen in normal subjects. Absence of dynein arms on ciliary peripheral microtubule doublets was a consistent finding in the patients' specimens and is suggested to be the basic defect in this syndrome that is responsible for immotility and absence of mucociliary clearance.

Adenosine Triphosphatases↗
PubMed1985

Ultrastructural observations on basal apparatus of respiratory cilia in immotile cilia syndrome.

The fine structure of the basal apparatus of cilia from respiratory epithelium has been studied in 5 subjects with immotile cilia syndrome without any specific defects in ciliary morphology. The ultrastructural analysis revealed the presence of abnormalities, heretofore undescribed, in basal bodies and accessory structures. In particular defective basal bodies of the so-called "half centriole" type, abnormal basal bodies showing an unusual dense granule in their central part, as well as abnormal accessory structures (i.e., giant striated roots, and double basal foot arising from single basal bodies) were found to be variously distributed in the patients examined. The significance of these morphological changes is discussed.

Adolescent↗
PubMed1981

Immotile-cilia syndrome and the cilia of the eye.

Ten patients with the immotile-cilia syndrome were subjected to an extensive eye examination, which failed to demonstrate any consistent single abnormality. The tests of the retinal function and the aqueous humor dynamics were normal. However, different corneal abnormalities--mostly without clinical significance--were observed in 9 out of 10 patients, suggesting a developmental disturbance.

Adult↗
PubMed1983

Regulation of ciliary pattern in dileptus (Ciliata). II. Formation of a cortical domain of sensory cilia from a domain of locomotor cilia.

The surface of the ciliate Dileptus contains locomotor and sensory domains. Each domain contains cilia of its own kind, arranged in a characteristic pattern. Transformation of a portion of the locomotor area into the sensory domain has been studied. Locomotor ciliary units in the transforming area undergo resorption of ciliary shafts, which are subsequently replaced by shafts of a sensory type. Anterior to each transforming ciliary unit a new one is formed, and both acquire sensory rootlets. As a result, a double-kinetosomal sensory unit emerges. A regulatory change in structure occurs over a definite area that is determined to become the sensory cortical domain.

Animals↗
PubMed1987

Aplasia of nasal cilia with situs inversus, azoospermia and normal sperm flagella: a unique variant of the immotile cilia syndrome.

A 23-year-old man with situs inversus and bronchiectasis was investigated because of azoospermia. Serum levels of testosterone and pituitary gonadotropins were normal. Scrotal exploration revealed no abnormalities of the testes, epididymides or vasa deferentia. Electron microscopy of a testicular biopsy specimen revealed a normal sperm flagellum structure, including the presence of dynein arms but 2 separate biopsies of the nasal mucosa revealed complete ciliary aplasia. This case, which bears a striking resemblance to Young's syndrome (bronchiectasis and azoospermia), represents a unique variant of the immotile cilia syndrome.

Adult↗
PubMed1996

The immotile cilia syndrome: characterization of the inflammatory response in nonsmoking patients with dysfunction of the cilia.

In order to demonstrate how patients with immotile cilia syndrome (ICS) are associated with lower respiratory tract inflammation, bronchoscopy and fractionated BAL were performed on eight ICS patients. Their VC was 84.5 +/- 16.7% (mean +/- SD) and FEV1 73.1 +/- 19.9% of predicted. Endobronchial signs of bronchitis were observed in all patients. The total cell concentrations in the BAL fluid were increased, compared to healthy nonsmokers (n = 10), both in the bronchial (BP) and alveolar portion (AP) (p < 0.01 for both). In the BP, this was mainly due to a high concentration of neutrophils (p < 0.001), whereas in the AP, the concentrations of lymphocytes (p < 0.01) as well as all types of granulocytes (p < 0.001-0.01) were elevated. The signs of active inflammation in the lower respiratory tract were confirmed by the concomitantly elevated (p < 0.001-0.05) concentrations of the soluble BAL components albumin, fibronectin and hyaluronan. Thus, the inflammatory response is not restricted to the ciliated conducting airways, but also occurs in the alveolar space and results-surprisingly-in only a slightly impaired lung function.

Adult↗
PubMed1984

Absence of cilia and basal bodies with predominance of brush cells in the respiratory mucosa from a patient with immotile cilia syndrome.

his report describes the ultrastructural alterations observed in the tracheal epithelium of a 13-year-old male with a history of recurrent pneumonia, chronic bronchitis, chronic otitis media, and situs inversus. The epithelium consisted of globlet and basal cells with many columnar cells that lacked cilia and basal bodies. The surface of these cells had regular microvilli and cytoplasmic features typical of brush cells.

Adolescent↗
PubMed1985

Genesis of cilia and microvilli of rat nasal epithelia during pre-natal development. II. Olfactory epithelium, a morphometric analysis.

Rat foetuses from intra-uterine days E14 through E22 (day before parturition) and adults were used for a quantitative scanning electron-microscopic examination of ciliogenesis in olfactory receptor cells and microvillogenesis in olfactory supporting cells. Four developmental stages in olfactory ciliogenesis can be discerned. Two of these are characterized by the presence of primary cilia only, the other two concern outgrowth in number and length of secondary cilia. (1) Primary cilia on undifferentiated cells; this stage occurs up to E14. (2) Primary cilia on differentiating olfactory receptor and also olfactory supporting cells. This stage begins at E14 and lasts, for the olfactory receptor cells, at least up to E22. On the supporting cells primary cilia are rarely observed after E18. Virtually all primary cilia are about 1 micron long. Up to E21 dendritic endings with primary cilia occur more frequently than those with any other number of cilia; all endings have a transitional stage in which they bear primary cilia only. (3) Secondary olfactory cilia increase in number. From E16 onwards the cells become multiciliated. Beginning at this stage and continuing up to E22 an average of one cilium per day is added to the endings. At E22 the average number of cilia observed per ending is about 70% of that in adults; more than 90% of the endings are multiciliated. From E15 to E22 the exchange rate between receptor cells with only primary cilia and multiciliated cells is about 0.5 X 10(6) cells/cm2 per day. When considered in the light of electrophysiological data on developing rats, our data suggest that when the cells have just primary cilia, they may respond indiscriminately to all odorants, whereas multiciliated cells display odorant specificity. (4) Secondary olfactory cilia increase in length. From E14 to E19 and over the whole population of receptor cells the cilia grow at an average rate of about 0.5 micron/day. Proximal parts of olfactory cilia are longer than primary cilia; olfactory cilia begin to taper in increasing numbers around E18. At E19 the receptive membrane surface, i.e. regions of the cells facing the nasal lumen, of individual cells is about 8%, and the increase in epithelial surface due to sprouting of cilia is 5% of such values in adult animals. Concomitant with the onset of tapering of olfactory cilia, i.e. around E18, microvilli of supporting cells show a spurt in growth from about 0.4 micron to about 1.3 micron. Unlike olfactory cilia they show no growth, on average, after E19.(ABSTRACT TRUNCATED AT 400 WORDS)

Animals↗
PubMed1985

Calcium-dependent phosphatidylinositol phosphorylation in lamellibranch gill lateral cilia.

Pure lateral (L) cilia may be separated from the remaining (R) cilia types of Mytilus edulis gill by serotonin activation after hypertonic shock. The two classes of cilia were permeabilized with 0.012% Triton X-100 and incubated with 32P-labeled ATP at low Ca++ (10(-7) M), where L cilia beat, or in high Ca++ (2-20 microM), where L cilia arrest but R cilia are active. The labeled cilia were separated into axoneme and membrane-matrix fractions by detergent extraction, subjected to SDS-PAGE on 5-15% gels, and autoradiographed. Neither cilia type undergoes Ca++-dependent phosphorylation of specific proteins, suggesting that neither Ca++-induced arrest in L cilia nor the Ca++ activation of other cilia is phosphorylation-dependent. However, lipid phosphorylation in L cilia is highly Ca++-dependent. Identified by thin-layer chromatography, the phospholipid that is phosphorylated in a Ca++-dependent manner is phosphatidylinositol 4-phosphate (PIP), yielding the 4,5-bisphosphate (PIP2). PIP2 increases at least 3-fold under Ca++-arrest conditions. Aequipecten gill lateral cilia, which require higher Ca++ levels for arrest, show even more striking changes. In both cases, the effect is maximal at micromolar Ca++ levels. Phosphorylation of other lipids is Ca++-independent. In the Ca++-insensitive or activated R cilia, PIP2 levels are intermediate, increasing only marginally with increased [Ca++]. The formation of PIP2 in response to Ca++, as opposed to its breakdown to form inositol 1,4,5-trisphosphate and diacylglycerol, may be characteristic of a Ca++ transport system. Mechanically sensitive, the L cilia arrest as a consequence of an inward flux of Ca++ ions, acting directly on the axoneme.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗