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At least 793 records · Page 44Linked to original sources
PubMed1995

Characterization of cilia-associated respiratory bacillus in rabbits and analysis of the 16S rRNA gene sequence.

The cilia-associated respiratory (CAR) bacillus is an unclassified, gram-negative bacterium that has been implicated as an etiologic agent of respiratory tract disease in laboratory rodents. A morphologically and antigenically similar organism has been identified in rabbits and is thought to be a related bacterium, although clinical signs of disease and histologic lesions are absent in infected rabbits. To compare the pathogenicity of rat- and rabbit-origin CAR bacillus isolates in rabbits, neonatal rabbits were experimentally infected with CAR bacillus isolates obtained from an infected rat and rabbit. Rabbits experimentally inoculated with rabbit-origin CAR bacillus had a nasal discharge, seroconverted and developed histologic lesions, whereas rabbits inoculated with rat-origin CAR bacillus seroconverted but did not have evidence of colonization of the respiratory tract. The CAR bacillus isolates were further examined at the genetic level by sequencing 1,261 base pairs of the 16S rRNA gene from six CAR bacillus isolates obtained from infected rabbits. A consensus sequence was obtained and compared with the analogous gene sequence data from rat-origin CAR bacillus isolates. Results indicated that these two organisms are distinctly different, with only 48.8% sequence homology. Comparison of the rabbit-origin 16S rRNA gene sequence with the database Genbank indicated that the organism is most closely related to members of the genus Helicobacter. Bacteria with the highest percentage of similarity with the rabbit-origin CAR bacillus were Helicobacter sp. strain Seymour and H. felis, with 91.1 and 90.8%, respectively. Findings of this study indicate that CAR bacillus isolates from rats and rabbits are host-specific and are different bacteria that belong to distinct genera.

Animals↗
PubMed1995

Calcium modulates the rapid kinetics of the odorant-induced cyclic AMP signal in rat olfactory cilia.

Although the cAMP and phosphoinositide (PI) second messenger systems are involved in olfactory signal transduction, aspects of their roles remain unclear. We have further examined the rapid kinetics of cAMP fluctuations in response to odorants in rat olfactory cilia isolated by calcium shock. Odorants cause a rapid and transient subsecond elevation of cAMP levels, as well as a more sustained signal lasting 5-10 sec. Basal cAMP levels demonstrate a biphasic calcium dependence; calcium enhanced both adenylyl cyclase (AC) and phosphodiesterase (PDE) activities. The odorant-induced cAMP response also demonstrated a biphasic dependence on calcium, with peak activity at 10 microM free calcium. All odorants tested were found to stimulate cAMP accumulation, and the dose-response curves were multiphasic, with less stimulation seen at higher concentrations. Dose-response curves performed for isovaleric acid at two free calcium concentrations demonstrated that calcium can influence cellular responsiveness to odorants and may be involved with signal potentiation as well as desensitization.

Animals↗
PubMed1994

Characterization of cilia-associated respiratory bacillus isolates from rats and rabbits.

Isolates of the cilia-associated respiratory (CAR) bacillus were harvested from the trachea of three naturally infected rats and five naturally infected rabbits and were grown on 3T3 mouse fibroblast cells. Isolates were compared by growth characteristics in mammalian cell culture, by use of protein and DNA analyses, and by experimental infections of BALB/c mice. Examination of CAR bacillus isolates by transmission electron microscopy indicated that organisms from rats and rabbits were similar in appearance and had an acidic mucopolysaccharide layer. In culture, the isolates from rats appeared larger than the rabbit isolates and formed large multiorganism aggregates, whereas isolates obtained from rabbits did not. Protein and antigenic analyses and DNA ribotyping revealed minor differences between isolates but could not be used to distinguish the rat from the rabbit isolates. Mice experimentally inoculated with CAR bacillus of rat origin developed interciliary colonization, seroconverted, and developed microscopic pulmonary lesions. Mice inoculated with isolates of rabbit origin did not display intraciliary colonization, seroconvert, or develop pulmonary disease. The findings of this study indicate that CAR bacillus isolates of rat and rabbit origins may be distinct strains and suggest that, in mice, isolates of rat origin may be more virulent than those of rabbit origin.

3T3 Cells↗
PubMed1995

Gold coating of respiratory cilia for scanning electron microscopy.

The optimal thickness of gold coating of cilia for scanning electron microscopy was studied using respiratory mucosa obtained from pigs. We tested 8 different coating times, from 10 seconds to 4 minutes, which resulted in gold layer thicknesses varying from 16 +/- 1 nm to 100 +/- 3 nm. The thickness of the gold layer with a coating time of 60 seconds and voltage of 2.5 kV was 43 +/- 5 nm. This thickness of gold layer gave good image quality without causing any electric charging. With thinner gold films, the amount of electric charging increased. When the coating time was longer, the gold layer was thicker but image quality did not improve. The thicknesses of the gold layers were measured using transmission electron microscopy (TEM).

Animals↗
PubMed1997

Sonographic survey of the upper abdomen in 10 families of patients with immotile cilia syndrome.

Ten patients affected by the immotile cilia syndrome (ICS) and their families received an ultrasound examination of the upper abdomen to observe the possible familial occurrence of biliary, pancreatic, and splenic alterations. In 9 patients the liver was localized in the left hypochondrium, and in 1 patient there was an accessory spleen. In all the examined kin, the subdiaphragmatic organs were normally positioned. Two parents had an accessory spleen. We conclude that the supposed increased frequency of polysplenia, asplenia, and biliary atresia among ICS subjects and their kin is overestimated and based only upon anecdotal reports.

Abdomen↗
PubMed1989

Immotile cilia syndrome including polysplenia, situs inversus, and extrahepatic biliary atresia.

Here we report on 2 sibs with immotile cilia syndrome (ICS), born to consanguineous Arab parents. Both had the Kartagener triad. In addition, one sib had polysplenia and extrahepatic biliary atresia. This observation, together with other literature reports, suggests that the occurrence of ICS with polysplenia and extrahepatic biliary atresia represent the result of a single dysmorphogenetic process. It is concluded that polysplenia and extrahepatic biliary atresia are rare and unusual manifestations of ICS.

Biliary Atresia↗
PubMed1994

The motilin gene: subregional localisation, tissue expression, DNA polymorphisms and exclusion as a candidate gene for the HLA-associated immotile cilia syndrome.

The product of the human motilin gene (MLN) has an important role in regulating gastrointestinal motility. The precise chromosomal localisation and expression of this gene are still unresolved. Here, we report a detailed study assigning MLN to 6p21.3; MLN is tightly linked to the HLA-DQalpha locus. Moreover, MLN expression has been evaluated in a large series of tissues. Positive signals have been obtained for brain, bronchi and a gastrointestinal malignancy. Direct sequencing exon by exon of the codifying region, intron/exon boundaries and promoter has allowed the identification of three DNA polymorphisms, one of which corresponds to a common protein variant. The chromosomal localisation of MLN, and its expression in broncoepithelial cells suggests that this gene is involved in immotile-cilia syndrome (ICS) disease. Sequence and segregation analysis of the MLN gene carried out in two families, in which the disease locus was previously assigned to 6p21.3, exclude MLN as a candidate gene for the HLA-associated form of ICS.

Base Sequence↗
PubMed1984

Immotile cilia syndrome: reduced chemotaxis and reduced number of intramembranous particles in granulocytes.

The neutrophil granulocytes of four patients with immotile cilia syndrome were investigated by means of freeze-fracture technique. Whereas most granulocytic functions (adherence, phagocytosis, killing of micro-organisms, reduction of NBT, and chemoluminescence) were in the normal range, chemotaxis of the neutrophils was clearly reduced; their plasma membrane revealed a profound reduction in the density of intramembrane particles. An interrelationship between reduced particle density and defective chemotaxis in neutrophils is assumed, but not yet proven.

Adult↗
PubMed1995

Calcitonin gene-related peptide in vitro stimulation of ciliary beat in human upper respiratory cilia.

Calcitonin gene-related peptide (CGRP) is one of the neuropeptides that is released in the human nasal mucosa after trigeminal nerve stimulation. We investigated the in vitro effect of CGRP on the ciliary beat frequency (CBF) of human upper respiratory cilia. Ciliated epithelium of freshly removed adenoidal tissues was used in all experiments. CBF was measured using a computerized photo-electric method. The results showed that CGRP exerted a significant dose-dependent stimulation on CBF in concentrations of 10(-9)-10(-6) M (n = 10), with a maximum of 23%. Our findings indicate that CGRP in vivo may help to protect the airway epithelium by directly stimulating ciliary beat.

Adenoids↗
PubMed1999

Genomic organization of the HSET locus and the possible association of HLA-linked genes with immotile cilia syndrome (ICS).

The kinesin-related protein (HSET) gene belongs to the kinesin superfamily, the members of which are involved in cellular transport processes. The HSET gene product was previously characterized by partial cDNA sequencing. The gene is located on the short arm of human Chromosome 6 (6p21.3), at the centromeric end of the major histocompatibility complex. Here, we report the genomic structure of the complete HSET gene together with its flanking loci. Sequence analysis of the 40 kilobase (kb) cosmid clone containing the HSET gene also revealed the presence of several new genes not related to the kinesin superfamily. These include a 60S ribosomal protein L35A-like pseudogene (rPL35A-like) on the telomeric side and a polycomb-like gene (PHF1), a copper tolerance-like gene (CUTA1) and the 5' part of the synaptic ras-GTPase-activating protein (SynGAP) gene centromeric of HSET. In addition, a complete 60S ribosomal protein L12-like (rPL12L) gene in intron 3 of the HSET gene was identified which appears to have an open reading frame. The possible involvement of the HSET gene and a beta-tubulin gene (TUBB) in the pathogenesis of immotile cilia syndrome (ICS) was studied by screening two unrelated ICS families with microtubular defects and suspected HLA linkage for mutations within the HSET gene and the TUBB gene. Four single base substitutions were detected in the HSET gene, and none in the TUBB gene. On the basis of these data, a role of the HSET and TUBB products in the pathogenesis of ICS in the two families is unlikely.

Amino Acid Sequence↗
PubMed1977

Tryptic fragmentation of 30-S dynein from Tetrahymena cilia.

30-S dynein ATPase from Tetrahymena cilia was digested with trypsin (dynein: trypsin = 20:1, by weight) at 25 degrees C for 20 min, resulting in the release of a 12-S fragment possessing ATPase activity. The 12-S ATPase fraction obtained by sucrose gradient centrifugation contained several polypeptide chains as indicated by SDS gel electrophoresis. The largest chain was smaller than the subunit of 30-S dynein and almost the same size as 14-S dynein. On the other hand, when 14-S dynein was digested in a similar manner, its sedimentation value changed from 14 to 12 S, but the peak of ATPase activity was retained at 14 S, suggesting differences in amino acid sequences between the 30 and 14-S dyneins. When the time course of tryptic digestion of 30-S dynein was investigated in a trypsin:dynein ratio of 1:200, discrete fragmentation took place, producing an intermediate fragment of 24 S and the 12-S fragment. The 24-S fragment recombined with outer fibers to some extent, while the 12-S fragment lacked this ability. However, the 12-S fragment was somewhat stimulated to recombine with outer fibers in the presence of other components involved in the trypsin digest. The enzymatic characteristics of the 12-S fraction were different from those of 30-S dynein, especially the activity dependence on pH showing a typical bell-shaped curve.

Adenosine Triphosphatases↗
PubMed1980

The immotile-cilia syndrome in a newborn infant.

A newborn infant was suspected to have the immotile-cilia syndrome. He had situs inversus, atelectasis and other pulmonary changes. A biopsy from the nasal mucosa at the age of 21 months confirmed the diagnosis and showed a lack of ciliary orientation and absent dynein arms. Chymotrypsin values, sweat test and most immunoglobulins were normal. The boy is now three years old. So far he has had rather mild respiratory symptoms. The importance of early diagnosis for management and prognosis is stressed.

Age Factors↗
PubMed1994

Ca2+ channels and signalling in cilia and flagella.

Ca(2+) plays a key role in the regulation of ciliary and flagellar movement. This article focuses on the initial steps of this regulation: how and where Ca(2+) enters cilia and flagella to trigger specific changes in axonemal motility. This knowledge is fundamental for understanding the sites, molecular targets and mechanisms of action of Ca(2+) within the cilium of flagellum.

Journal Article↗
PubMed1994

Primary ciliary dyskinesia (the immotile cilia syndrome).

OBJECTIVE: The purpose of this review is to familiarize the reader with the genetic aspects, clinical manifestations, diagnostic techniques and management of the primary ciliary dyskinesia syndrome. Further, this article illustrates some unusual features of this syndrome and discusses some speculative hypotheses concerning its pathogenesis and clinical presentation. DATA SOURCES: The bibliography includes references in English as well as some references of historical interest in German. Both human and veterinary literature are quoted. Sources included computerized bibliographic searches of recent literature and reviews of literature. STUDY SELECTION: Selection of papers was made based on their historic importance in the definition and characterization of the disease, and on reviews of large bodies of novel or interesting information. Some review papers were not included to avoid repetition. RESULTS: Although the incidence of primary ciliary dyskinesia is low, the inclusion of this condition in the differential diagnosis of chronic and recurrent sinobronchial disease in children and older individuals is very common. Primary ciliary dyskinesia should be suspected in individuals who present chronic respiratory symptoms already in the neonatal period, develop profuse, chronic mucopurulent rhinorrhea, and chronic otitis media and sinusitis. Chronic cough, obstructive lung disease, and bronchorrhea associated with the aforementioned manifestations should also make clinicians suspect this syndrome. Male sterility is almost universally present and situs inversus is present in 50% of affected persons. The diagnosis of primary ciliary dyskinesia is clinical and is confirmed by studies of ciliary motility and ultrastructure of the respiratory mucosa. Management is directed to microbial suppression by frequent antibiotic administration, and to clearing of retained secretions. CONCLUSIONS: The diagnosis of primary ciliary dyskinesia requires familiarity with the clinical picture and the specific techniques of identification. Although the basic mechanism of disease is known, the molecular genetics of primary ciliary dyskinesia and the causes for the phenotypic variability remain to be explained. Future research should be directed to the identification of the gene(s) responsible for the manifestations of the disease and to effective methods of activation, in vivo, of dysfunctional cilia.

Ciliary Motility Disorders↗
PubMed1980

Necrospermia or immotile cilia syndrome as a cause of male infertility.

A case of infertility secondary to necrospermia was reinvestigated and reclassified as the "immotile cilia syndrome". The immotile spermatozoa were viable but motionless owing to an ultrastructural defect of the tail characterized by absence of microtubular dynein sidearms. There is no therapy for this congenital disorder and artificial insemination was performed.

Adult↗
PubMed2003

Enhanced blood compatibility of polymers grafted by sulfonated PEO via a negative cilia concept.

In our laboratory sulfonated PEO (PEO-SO(3)) was designed as a "negative cilia model" to investigate a synergistic effect of PEO and negatively charged SO(3) groups. PEO-SO(3) itself exhibited a heparin-like anticoagulant activity of 14% of free heparin. Polyurethane grafted with PEO-SO(3) (PU-PEO-SO(3)) increased the albumin adsorption to a great extent but suppressed other proteins, while PU-PEO decreased the adsorption of all the proteins. The platelet adhesion was decreased on PU-PEO but least on PU-PEO-SO(3) to demonstrate an additional effect of SO(3) groups. The enhanced blood compatibility of PU-PEO-SO(3) in the ex vivo rabbit and in vivo canine implanting tests was confirmed. Furthermore, PU-PEO-SO(3) exhibited an improved biostability and suppressed calcification in addition to the enhanced antithrombogenicity. The in vivo antithrombogenicity and biostability were improved in the order of PU PU-PEO>PU-PEO-SO(3) in spite of the possible attraction between negative SO(3) groups and positive calcium ions. The bioprosthetic tissue (BT) was grafted with H(2)N-PEO-SO(3) via glutaraldehyde (GA) residues after conventional GA fixation. BT-PEO-SO(3) also displayed the decreased calcification by in vivo animal models. The application of PEO-SO(3) was extended by designing amphiphilic copolymers containing PEO-SO(3) moiety and hydrophobic long alkyl groups as anchors. The superior effect of PEO-SO(3) groups on thromboresistance compared to PEO was confirmed also in the case of copolymers coated or blended with other polymers and the systems coupled by UV irradiation, photoreaction or gold/sulfur or silane coupling technology, and therefore it might be very useful for the medical devices.

Adsorption↗