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At least 325 records · Page 18Linked to original sources
PubMed1997

[Observation on postmortem tracheal cilia of drowning by scanning electron microscope (SEM)].

The changes of tracheal cilia of drowning rat were observed by SEM at 0, 24, 48, 72, 96 h after death. The authors found: the tracheal cilia lost their normal arrangement in some area, and the opening increased on the top of the Goblet cell. There were some thing resembling cotton, or looking like network on the surface of tracheal cilia. At different times after death, the tracheal cilia appeared adhering, downing, and destroying, Goblet cell membrane creasing, the top of cell flattening and the cell collapsing. Observing this changes, we could estimate the time of death.

Animals↗
PubMed1989

[A case of immotile cilia syndrome and a review of Japanese cases].

A case of immotile cilia syndrome (ICS) is presented. A 34-year-old male, who had suffered from recurrent bronchitis, sinusitis and otitis media since early childhood, was admitted to Keio University Hospital complaining of productive cough and infertility. A saccharin test showed prolonged nasal clearance time, and semen analysis revealed immotile sperm. By electron microscopic observation of cilia of the nasal mucous epithelium and the sperm, inner and outer dynein arm defect, abnormal microtubular arrangement and compound cilia were detected and he was diagnosed as ICS. Thirty eight Japanese cases of ICS collected from the literature were analyzed concerning clinical manifestations, airway clearance, family history and ciliary ultrastructural abnormalities (Table 2, 3). Recurrent bronchitis, male infertility, chronic sinusitis, otitis and decrease in airway clearance were very common in these patients. Female infertility was more common than anticipated. The prevalence rate of situs inversus was more than 50%, probably due to more attention having been paid to Kartagener's syndrome in Japan. Recent studies show that the cilia of this syndrome is not always immotile but characterized by hypomotility or asynchrony, and have attempted to determine the relationship between each ciliary ultrastructural abnormality and motility pattern. It is necessary that more patients including incomplete and mild cases should be investigated.

Adult↗
PubMed1985

Abnormal length of cilia as a possible cause of defective mucociliary clearance.

A 12-year-old girl with obstructive lung disease was examined with respect to the ultrastructure of her nasal mucociliate epithelium. She had the characteristics of the immotile-cilia syndrome: chronic rhinitis, sinusitis, and bronchitis, a severely decreased mucociliary clearance of the lungs, and nasal polyps. The nasal cilia showed a normal cross-sectional appearance but were found to have twice the normal length: 12 micron rather than 6 micron. We conclude that the patient represents a new subgroup of the immotile-cilia syndrome and that her cilia are unable to perform their normal tasks.

Bronchitis↗
PubMed1983

Nasal cilia in normal man, primary ciliary dyskinesia and other respiratory diseases: analysis of motility and ultrastructure.

We studied the ultrastructure and function of nasal cilia from non-atopic and atopic non-smokers, smokers, patients with chronic rhinitis and cystic fibrosis, and compared these to cilia from patients with primary ciliary dyskinesia (PCD). Atypical cilia were found in normal subjects and patients with a variety of chronic respiratory tract diseases, but the proportion was small (less than 5%). Ciliary wave form, beat frequency, and mucociliary transport were normal. In patients with PCD, the proportion of atypical cilia and ultrastructural abnormalities was significantly greater than normal subjects and patients without PCD p less than 0.0001. Beat frequency was reduced, ciliary wave form was grossly abnormal, and mucociliary transport was virtually absent. These findings suggest that ciliary ultrastructural abnormalities are related to altered ciliary function and have aetiological relevance in the generalized chronic respiratory tract disease of PCD.

Cilia↗
PubMed1984

[Immotile cilia disease with neonatal disclosure. Ultrastructural study].

Recurrent bronchopulmonary and E.N.T. infections in a 2-month old child with complete situs inversus suggested an immotile cilia syndrome. Electron microscopy of the respiratory epithelium cilia demonstrated an ultra structural abnormality (defective radial spokes) typical of this recently discovered syndrome. This case is similar to 3 other cases in infants reported in the literature. It shows that the clinical manifestations of ciliary dysfunction may occur soon after birth and that early detection is desirable for optimal treatment. The type of abnormality detected and the percentage of cilia affected demonstrate that the syndrome is congenital and not acquired and provide information of the degree of ciliary dyskinesia. The genetic aspects are discussed. A diagnosis of "immotile cilia" syndrome should systematically be envisaged in infants with recurrent pneumonia or otitis of unknown aetiology, or when the clinical context (situs inversus, family history) is suggestive of the conditions.

Bronchi↗
PubMed1983

Phosphorylation of endogenous proteins of cilia from Paramecium tetraurelia in vitro.

Several endogenous substrate proteins of cilia from axenically grown Paramecium tetraurelia were phosphorylated in vitro by inherent protein kinases (PKs). Labeling was stimulated by cAMP and to a lesser extent by cGMP. ATP breakdown was most rapid in cilia and subciliary fractions. Using multiple substrate additions during incubations it was shown that phosphorylation was almost completed within 30 s. Very little dephosphorylation by phosphoprotein phosphatases occurred during 5 min of incubation. Proteins of molecular weight of 103 000 and 46 000 were shown to be particularly associated with axonemal structures of the cilia. No distinct differences in phosphorylation patterns were apparent in ciliary membrane vesicles of low and high buoyant density, which exhibit differential enzyme patterns. cAMP receptor proteins were identified by use of the photoaffinity label 8-azido-[32P]cAMP. Receptor proteins with apparent molecular weights of 43 000, 39 000, 37 000, 31 000 and 30 000 were probably related to the regulatory subunits of cAMP-dependent protein kinases as evidenced by inhibition of incorporation of the photoaffinity label by low concentrations of cAMP. Tagging of a protein of 85 000 molecular weight was specifically inhibited by cGMP, thus in all likelihood it corresponded to a cGMP-dependent protein kinase. Corresponding autophosphorylated protein bands were observed with gamma-[32P]ATP. A functional role for protein phosphorylation in cilia of Paramecium remains to be established.

Animals↗
PubMed1980

Ultrastructural defects of respiratory tract cilia associated with chronic infections.

Ultrastructural defects were demonstrated in nasal and bronchial cilia from a 12-year-old boy with repeated upper and lower respiratory tract infections. Numerous abnormalities were found, including single axonemes surrounded by excess cytoplasmic matrix, compound cilia, intracytoplasmic microtubular doublets, and cilia contained within a periciliary sheath. Dynein arms were missing from the majority of the peripheral microtubular doublets. The most striking abnormality, however, was a disorientation of cilia as judged by the increased variation in the orientation of central microtubules. Because of these ultrastructural abnormalities, it is highly likely that ciliary motility was markedly decreased and that defective mucociliary transport was responsible for chronic and repeated upper respiratory tract infections.

Bronchi↗
PubMed1981

Clinical expressions of immotile cilia syndrome.

To date three distinct morphologic types of immotile cilia disorder in man have been recognized. Patients with the disorder have variable clinical manifestations of disease of the upper and lower respiratory tract. Twenty-one patients with immotile cilia syndrome have been evaluated from the standpoint of history, clinical presentation, radiologic changes, pulmonary function tests, and mucociliary clearance. The results have been compared to the pathologic changes noted on ultrastructural examination of the cilia. Although clinical severity does not correlate with the type of abnormality found, a profile of clinical signs and symptoms suggestive of immotile cilia syndrome has emerged. The triad of productive cough, sinusitis, and otitis was a consistent finding in all. Situs inversus occurred in only half of the patients.

Adolescent↗
PubMed1981

Genetical and ultrastructural aspects of the immotile-cilia syndrome.

The immotile-cilia syndrome is a congenital disorder characterized by all cilia in the body being either immotile or showing an ineffective beating pattern. Most symptoms, not unexpectedly, come from the ciliated epithelia, but two further symptoms are: (1) male sterility caused by the spermatozoa being unable to swim progressively (the sperm tail has the same structure as a cilium), and (2) situs inversus in 50% of the cases possible caused by an inability of embryonic cilia to shift the heart to the left side. By electron microscopy, one can see directly which of the many ciliary components is the missing one. The molecular basis of this congenital defect can then be detected, and it has been found to be a heterogeneous disease. There are many genes that, when mutated, will cause the cilia to be dysfunctional or totally immotile. The fact that many genes may be responsible for the syndrome will also explain why it has a relatively high prevalence and why previous investigators have been unable to locate the (assumed single) gene by linkage analysis. The trait, situs inversus, is of particular interest as it occurs in only 50% of the assumed homozygotes. I conclude that the wild-type genes code for a control of the proper body asymmetry and the mutated ones for a lack of control, and, hence, to a random situs determination.

Animals↗
PubMed1976

Abnormal cilia in human bronchial epithelium.

Abnormal cilia are a common occurrence in human bronchial epithelium. Bronchial specimens were obtained at surgery or at "immediate autopsy" from patients with neoplastic and nonneoplastic diseases; these patients included smokers and one known nonsmoker. Areas of bronchial epithelium far removed from the tumor mass were studied. Ciliary abnormalities fell into four major categories: (1) cilia with a single axoneme and excess cytoplasmic matrix; (2) compound cilia; (3) intracytoplasmic microtubular doublets; and (4) cilia within periciliary sheaths.

Adenocarcinoma↗
PubMedDate not supplied

Transmission electron microscopic demonstration of abnormalities on the tracheal cilia of chicks exposed to formaldehyde during hatching.

Formaldehyde gas used to fumigate hatcheries for control of microbial contamination has an adverse effect on tracheal cilia function and morphology. Evaluation of the changes revealed alterations in the ultrastructure of the axoneme with the absence of B subfibers and the production of additional A subfibers. Spikes and vesicular blebs in the cilia walls were evident in formaldehyde-exposed cilia. These changes could result in ciliastasis and cilia loss.

Animals↗
PubMed1993

Intraocular cilia. Report of six cases of 6 weeks' to 32 years' duration.

In this study, we describe the clinicopathologic features seen in six cases of intraocular cilia and review the features of previously reported cases. Ultrastructural studies of the cilia showed partial loss of the cuticle layer, diffuse accumulation of small electron-dense granules in cuticle and cortical cells, and loss of continuous cell membranes of the cortex. The cilia were almost completely intact in most of our cases. Intraocular cilia can be tolerated for long periods.

Adolescent↗
PubMed2005

The mouse ortholog of EFHC1 implicated in juvenile myoclonic epilepsy is an axonemal protein widely conserved among organisms with motile cilia and flagella.

The gene product of EFHC1 recently implicated in juvenile myoclonic epilepsy (JME) was found to be a homolog of Chlamydomonas axonemal protein Rib72, whose homologs are present in a wide variety of organisms that have motile cilia and flagella. Western blot analyses and immunofluorescence localization of the mouse ortholog mRib72-1/Efhc1 indicated that it is indeed abundantly present in sperm flagella and tracheal cilia but only in a small amount in the brain. It is not present in immotile primary cilia. These observations raise the possibility that malfunction of motile cilia is involved in the development of JME.

Animals↗
PubMed1997

Therapeutic approach of immotile cilia syndrome by intracytoplasmic sperm injection: a case report.

OBJECTIVE: To present a case of immotile cilia syndrome, a very rare cause of male infertility and to evaluate the role of the recently suggested treatment by intracytoplasmic sperm injection (ICSI). DESIGN: Case report. SETTING: Tertiary-care academic hospital. PATIENT(S): One man with immotile cilia syndrome, showing no motile spermatozoa despite normal morphology and viability. INTERVENTION(S): The patient's partner underwent two cycles with IVF of the oocytes achieved by ICSI. MAIN OUTCOME MEASURE(S): Evaluation of ICSI procedure in cases of immotile cilia syndrome. RESULT(S): Thirty-three percent of the oocytes were fertilized and subsequently divided enabling ET in both cycles. CONCLUSION(S): Intracytoplasmic sperm injection seems to represent a promising approach to the problem of infertility in men with immotile cilia syndrome.

Adult↗
PubMed1994

Ciliostasis and loss of cilia induced by Mycoplasma hyopneumoniae in porcine tracheal organ cultures.

In vivo- and in vitro-grown Mycoplasma hyopneumoniae organisms were inoculated onto newborn piglet tracheal organ cultures to provide a model for interaction of this organism with ciliated respiratory epithelium. Ciliostasis and loss of cilia in tracheal rings were induced by M. hyopneumoniae grown in vivo and with low-passage cultures when grown in vitro. Levels of calmodulin or dehydrogenase enzymes in tracheal ring epithelium were not altered even though ciliostasis and loss of cilia induced by M. hyopneumoniae were extensive. The capacity for inducing epithelial damage diminished with in vitro passage of the organism. Attempts to induce higher-passage cultures to attach to cilia, cause ciliostasis, or cause ciliary damage by supplementation of mycoplasmal medium with porcine lung extract failed. Epithelial damage induced by M. hyopneumoniae in tracheal rings was averted by using porcine immune serum or by separating the organisms from ciliated epithelium with a 0.1-microns-pore-size membrane. Attachment, or at least close association, of M. hyopneumoniae to ciliated epithelium appeared to be necessary to induce ciliostasis and loss of cilia in this model.

Animals↗
PubMed2005

Gli2 and Gli3 localize to cilia and require the intraflagellar transport protein polaris for processing and function.

Intraflagellar transport (IFT) proteins are essential for cilia assembly and have recently been associated with a number of developmental processes, such as left-right axis specification and limb and neural tube patterning. Genetic studies indicate that IFT proteins are required for Sonic hedgehog (Shh) signaling downstream of the Smoothened and Patched membrane proteins but upstream of the Glioma (Gli) transcription factors. However, the role that IFT proteins play in transduction of Shh signaling and the importance of cilia in this process remain unknown. Here we provide insights into the mechanism by which defects in an IFT protein, Tg737/Polaris, affect Shh signaling in the murine limb bud. Our data show that loss of Tg737 results in altered Gli3 processing that abrogates Gli3-mediated repression of Gli1 transcriptional activity. In contrast to the conclusions drawn from genetic analysis, the activity of Gli1 and truncated forms of Gli3 (Gli3R) are unaffected in Tg737 mutants at the molecular level, indicating that Tg737/Polaris is differentially involved in specific activities of the Gli proteins. Most important, a negative regulator of Shh signaling, Suppressor of fused, and the three full-length Gli transcription factors localize to the distal tip of cilia in addition to the nucleus. Thus, our data support a model where cilia have a direct role in Gli processing and Shh signal transduction.

Animals↗
PubMed1991

[A case of immotile cilia syndrome accompanied by retinitis pigmentosa].

A case of immotile cilia syndrome accompanied by retinitis pigmentosa is reported. This syndrome involves congenital ciliary ultrastructural abnormality. A 27-year-old male complained of repeated pneumonia, sinusitis, and middle otitis. In addition, he had sperm motor insufficiency and electron microscopic finding of cilia led to the diagnosis of the present syndrome. Both fundi presented remarkable degeneration of retinal pigment epithelium and choroid and marked arterial narrowing. Constriction of the visual field and extinguished ERG were also noted. Abnormality of cilia of the retinal pigment epithelium was suggested. It was proposed that retinitis pigmentosa may be caused by abnormal cilia of the retinal pigment epithelium.

Adult↗
PubMed1984

Electron microscopic analysis of the effect of progesterone upon the hormone-sensitive solitary cilia and centriolar complexes in the luminal epithelial cells of the uterus of the ovariectomized-adrenalectomized rat.

The time course of the hormonally controlled deciliation cycles of the centriolar complexes in the cells of the luminal epithelium of the uterus of the ovariectomized and adrenalectomized rat was analyzed at ultrastructural levels. The results were expressed quantitatively. The half-life of the solitary cilia after progesterone administration in the cell population was 14.6 hr, longer approximately by 1 hr than that previously reported for the estrogen-induced deciliation of the same cells. The time course of the progesterone-induced loss of solitary cilia strongly suggested that the phenomenon is biphasic. After the initial loss of 50%, the process progressed slowly. Twenty-four hours after the injection of the hormone, 35% of the cilia remained; after 60 hours, 4% were still present. This is in striking contrast to the effect of estrogen, which causes almost complete deciliation within 24 hr after the injection of the hormone. Prolonged exposure of the deciliated luminal epithelial cells to progesterone leads to disarrangement of the diplosome relationship. The phenomenon might be causally correlated with the block of estrogen-induced mitoses by this hormone; this view, however, needs further experimental corroboration.

Adrenalectomy↗