Clinical care of children with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Lucas, Jane S; Alanin, Mikkel Christian; Collins, Samuel

2017-01-01

INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare heterogeneous disorder, usually inherited as an autosomal recessive condition but X-linked inheritance is also described. Abnormal ciliary function in childhood leads to neonatal respiratory distress in term infants, persistent wet cough...... is inappropriate since differences in pathophysiology, morbidity and prognosis risk treatment failure and lack of adherence. Areas covered: Review authors searched PubMed and Cochrane databases for publications relating to management of children with PCD. Because of the paucity of data, we emphasise the need...

Progressive hemifacial atrophy with ciliary body atrophy and ocular hypotony

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T Ashwini Kini

2015-01-01

Full Text Available Progressive hemifacial atrophy (PHA is a disease of unknown etiology affecting one-half of the face. Ocular involvement is uncommon. Atrophy of iris is rare, with only a few cases of partial atrophy being reported in the literature. We report a case of total atrophy of iris and ciliary body with associated ocular hypotony in a 16-year-old girl with PHA. We believe this is the first reported case of complete atrophy of iris and ciliary body in PHA. Ocular hypotony in PHA was thought to be due to intra-ocular inflammation. However in our case it appears to be secondary to severe atrophy of the ciliary body.

Airway ciliary dysfunction and respiratory symptoms in patients with transposition of the great arteries.

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Zahid, Maliha; Bais, Abha; Tian, Xin; Devine, William; Lee, Dong Ming; Yau, Cyrus; Sonnenberg, Daniel; Beerman, Lee; Khalifa, Omar; Lo, Cecilia W

2018-01-01

Our prior work on congenital heart disease (CHD) with heterotaxy, a birth defect involving randomized left-right patterning, has shown an association of a high prevalence of airway ciliary dysfunction (CD; 18/43 or 42%) with increased respiratory symptoms. Furthermore, heterotaxy patients with ciliary dysfunction were shown to have more postsurgical pulmonary morbidities. These findings are likely a reflection of the common role of motile cilia in both airway clearance and left-right patterning. As CHD comprising transposition of the great arteries (TGA) is commonly thought to involve disturbance of left-right patterning, especially L-TGA with left-right ventricular inversion, we hypothesize CHD patients with transposition of great arteries (TGA) may have high prevalence of airway CD with increased respiratory symptoms. We recruited 75 CHD patients with isolated TGA, 28% L and 72% D-TGA. Patients were assessed using two tests typically used for evaluating airway ciliary dysfunction in patients with primary ciliary dyskinesia (PCD), a recessive sinopulmonary disease caused by respiratory ciliary dysfunction. This entailed the measurement of nasal nitric oxide (nNO), which is typically low with PCD. We also obtained nasal scrapes and conducted videomicroscopy to assess respiratory ciliary motion (CM). We observed low nNO in 29% of the patients, and abnormal CM in 57%, with 22% showing both low nNO and abnormal CM. No difference was observed for the prevalence of either low nNO or abnormal ciliary motion between patients with D vs. L-TGA. Respiratory symptoms were increased with abnormal CM, but not low nNO. Sequencing analysis showed no compound heterozygous or homozygous mutations in 39 genes known to cause PCD, nor in CFTR, gene causing cystic fibrosis. As both are recessive disorders, these results indicate TGA patients with ciliary dysfunction do not have PCD or cystic fibrosis (which can cause low nNO or abnormal ciliary motion). TGA patients have high

ICK is essential for cell type-specific ciliogenesis and the regulation of ciliary transport.

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Chaya, Taro; Omori, Yoshihiro; Kuwahara, Ryusuke; Furukawa, Takahisa

2014-06-02

Cilia and flagella are formed and maintained by intraflagellar transport (IFT) and play important roles in sensing and moving across species. At the distal tip of the cilia/flagella, IFT complexes turn around to switch from anterograde to retrograde transport; however, the underlying regulatory mechanism is unclear. Here, we identified ICK localization at the tip of cilia as a regulator of ciliary transport. In ICK-deficient mice, we found ciliary defects in neuronal progenitor cells with Hedgehog signal defects. ICK-deficient cells formed cilia with mislocalized Hedgehog signaling components. Loss of ICK caused the accumulation of IFT-A, IFT-B, and BBSome components at the ciliary tips. In contrast, overexpression of ICK induced the strong accumulation of IFT-B, but not IFT-A or BBSome components at ciliary tips. In addition, ICK directly phosphorylated Kif3a, while inhibition of this Kif3a phosphorylation affected ciliary formation. Our results suggest that ICK is a Kif3a kinase and essential for proper ciliogenesis in development by regulating ciliary transport at the tip of cilia. © 2014 The Authors.

Melanocytoma of the ciliary body misdiagnosed as iridodialysis

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Kim M

2014-05-01

Full Text Available Moosang Kim, Seung-Jun LeeDepartment of Ophthalmology, School of Medicine, Kangwon National University, Chuncheon, Republic of KoreaAbstract: A 62-year-old female presented to our institution with dimness of vision in her right eye. On examination, her best corrected visual acuity was 20/100 in the right eye. The intraocular pressures were 14 mmHg in both eyes. Slit-lamp examination revealed nuclear sclerotic cataracts bilaterally and iridodialysis in her right eye. Seven days after the first visit, cataract surgery was performed without any complications. One year later, she presented to our institution with acute visual loss and ocular pain in the right eye. Best corrected visual acuity of the right eye was light perception and the intraocular pressure was 44 mmHg. Slit-lamp examination revealed a ciliary body mass with widespread pigment dispersion in the anterior segment. Due to no useful vision and uncontrolled pain, enucleation of the right eye was performed. Histopathologic examination revealed a melanocytoma of the ciliary body.Keywords: ciliary body, iridodialysis, melanocytoma

Growth of the crabgrass species Digitaria ciliaris and Digitaria nuda Crescimento das espécies de capim-colchão Digitaria ciliaris e Digitaria nuda

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R.C. Souza

2012-06-01

Full Text Available The aim of this research paper was to compare the growth of D. ciliaris and D. nuda crabgrass species under non-competitive conditions. To this end, two experiments were conducted, one from March - July 2010 and the other from February - June 2011. The experimental design of both trials was completely randomized making a factorial (2 seasons x 2 species crabgrass x 12 evaluation periods with four replications. Assessments began at 15 days after sowing (DAS, and repeated weekly until 92 DAS. The variables evaluated were total dry matter (roots+leaves+stems, leaf area, leaf number and tiller. The results were submitted to analysis of variance and the absolute growth rate, relative growth rate and leaf area ratio were calculated using the means, which were adjusted regression models. The crabgrass species were significantly different in leaf area, leaf number, tiller number and dry matter per plant. D. ciliaris for all variables was statistically higher than D. nuda. Regarding the speed at which the growth of the species occurred, the absolute growth rate and relative growth rate of D. ciliaris was also greater than D. nuda. In addition, D. ciliaris also had a lower leaf area ratio indicating greater efficiency in converting light energy into carbohydrates. It can be concluded that D. ciliaris has a higher growth rate in conditions where there is no limitation of nutrients and water availability in relation to D. nuda, mainly due to D. ciliaris have greater leaf area, number of leaves and dry matter accumulation per plant.O objetivo da presente pesquisa foi comparar o crescimento das espécies de capim colchão D. ciliaris e D. nuda, em condições não-competitivas. Para isso, foram conduzidos dois experimentos, um de março a julho de 2010 e outro de fevereiro a junho de 2011. O delineamento experimental de ambos os ensaios foi inteiramente casualizado, perfazendo um esquema fatorial (2 épocas x 2 espécies de capim colchão x 12 períodos de

Oligodendroglioma of the ciliary body: a unique case report and the review of literature

International Nuclear Information System (INIS)

Guo, Qing; Hao, Jie; Sun, Shou bin; Xu, Shou ping; Yang, Qian; Guo, Qi liang; Cui, Guo dong

2010-01-01

To date, there is no report in the international literature of an oligodendroglioma of the ciliary body, nor is there an analysis of the possible origins of this lesion. Here we report on a 52-year-old man admitted to our hospital with a ciliary body tumor revealed by clinical examination and ultrasound, computed tomography and magnetic resonance imaging studies. Following enucleation, pathological and immunohistochemical analyses were performed. Postoperative histopathological staining results included OLIGO-2(+) and GFAP(-), leading to a pathological diagnosis of oligodendroglioma of the ciliary body in the right eye (WHO grade II). Since malignant gliomas derive from transformed neural stem cells, the presence of oligodendroglioma in the ciliary body supports the hypothesis that gliomas can occur wherever neural stem cells exist. Tumors of the ciliary body derived from oligodendrocytes are difficult to diagnose; pathological analyses are essential

Circumferential Ciliary Body Cysts Presenting as Acute Pigment Dispersion and Ocular Hypertension.

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Sarıgül Sezenöz, Almila; Güngör, Sirel Gür; Kıratlı, Hayyam; Akman, Ahmet

2017-09-15

To report a case of circumferential neuroepithelial cyst of the ciliary body presenting with pigment dispersion (PD) and ocular hypertension. 48-year-old female patient presented with a complaint of pain in the left eye. On examination, visual acuity of the left eye was 0.9, and the intraocular pressure was 48 mmHg. Biomicroscopic anterior segment examination of the left eye revealed 4+ pigmented cells in the anterior chamber. Active PD from the pupillary region at 11 o'clock was noticed at the time of the examination. Ultrasound biomicroscopy demonstrated 360º cystic lesions of the ciliary body in the left eye. The patient was diagnosed as neuroepithelial cyst of the ciliary body. Our case is unique as it is the first case of circumferential neuroepithelial ciliary body cyst presenting with acute PD and ocular hypertension.

Cellular Mechanisms of Ciliary Length Control

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Jacob Keeling

2016-01-01

Full Text Available Cilia and flagella are evolutionarily conserved, membrane-bound, microtubule-based organelles on the surface of most eukaryotic cells. They play important roles in coordinating a variety of signaling pathways during growth, development, cell mobility, and tissue homeostasis. Defects in ciliary structure or function are associated with multiple human disorders called ciliopathies. These diseases affect diverse tissues, including, but not limited to the eyes, kidneys, brain, and lungs. Many processes must be coordinated simultaneously in order to initiate ciliogenesis. These include cell cycle, vesicular trafficking, and axonemal extension. Centrioles play a central role in both cell cycle progression and ciliogenesis, making the transition between basal bodies and mitotic spindle organizers integral to both processes. The maturation of centrioles involves a functional shift from cell division toward cilium nucleation which takes place concurrently with its migration and fusion to the plasma membrane. Several proteinaceous structures of the distal appendages in mother centrioles are required for this docking process. Ciliary assembly and maintenance requires a precise balance between two indispensable processes; so called assembly and disassembly. The interplay between them determines the length of the resulting cilia. These processes require a highly conserved transport system to provide the necessary substances at the tips of the cilia and to recycle ciliary turnover products to the base using a based microtubule intraflagellar transport (IFT system. In this review; we discuss the stages of ciliogenesis as well as mechanisms controlling the lengths of assembled cilia.

Ciliary body toxicities of systemic oxcarbazepine and valproic acid treatments: electron microscopic study.

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Göktaş, Güleser; Aktaş, Zeynep; Erdoğan, Deniz; Seymen, Cemile Merve; Karaca, Emine Esra; Cansu, Ali; Serdaroğlu, Ayşe; Kaplanoğlu, Gülnur Take

2015-01-01

Ciliary body is responsible for humour aqueous production in posterior chamber. Valproic acid (VPA) has been widely used for the treatment of epilepsy and other neuropsychiatric diseases such as bipolar disease and major depression. Oxcarbazepine (OXC) is a new anti-epileptic agent that has been used recently for childhood epilepsies such as VPA. In this study, we aimed to investigate the effects of VPA and OXC treatments used as antiepileptic in ciliary body by electron microscopy. In our study, 40 Wistar rats (21 days old) were divided equally into four groups which were applied saline (group 1), VPA (group 2), OXC (group 3) and VPA + OXC (group 4). The as-prepared ocular tissues were characterized by transmission electron microscopy (TEM) technique in scanning and transmission electron microscopy (SEM-TEM) (Carl Zeiss EVO LS10). The results confirmed that VPA caused dense ciliary body degeneration. Additionally, ciliary body degeneration in group 4 was supposed to be due to VPA treatment. Ciliary body damage and secondary outcomes should be considered in patients with long-term VPA therapy.

cAMP Stimulates Transepithelial Short-Circuit Current and Fluid Transport Across Porcine Ciliary Epithelium.

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Cheng, Angela King-Wah; Civan, Mortimer M; To, Chi-Ho; Do, Chi-Wai

2016-12-01

To investigate the effects of cAMP on transepithelial electrical parameters and fluid transport across porcine ciliary epithelium. Transepithelial electrical parameters were determined by mounting freshly isolated porcine ciliary epithelium in a modified Ussing chamber. Similarly, fluid movement across intact ciliary body was measured with a custom-made fluid flow chamber. Addition of 1, 10, and 100 μM 8-Br-cAMP (cAMP) to the aqueous side (nonpigmented ciliary epithelium, NPE) induced a sustained increase in short-circuit current (Isc). Addition of niflumic acid (NFA) to the aqueous surface effectively blocked the cAMP-induced Isc stimulation. The administration of cAMP to the stromal side (pigmented ciliary epithelium, PE) triggered a significant stimulation of Isc only at 100 μM. No additive effect was observed with bilateral application of cAMP. Likewise, forskolin caused a significant stimulation of Isc when applied to the aqueous side. Concomitantly, cAMP and forskolin increased fluid transport across porcine ciliary epithelium, and this stimulation was effectively inhibited by aqueous NFA. Depleting Cl- in the bathing solution abolished the baseline Isc and inhibited the subsequent stimulation by cAMP. Pretreatment with protein kinase A (PKA) blockers (H89/KT5720) significantly inhibited the cAMP- and forskolin-induced Isc responses. Our results suggest that cAMP triggers a sustained stimulation of Cl- and fluid transport across porcine ciliary epithelium; Cl- channels in the NPE cells are potentially a cellular site for this PKA-sensitive cAMP-mediated response.

Photoreceptor Sensory Cilium: Traversing the Ciliary Gate

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Hemant Khanna

2015-10-01

Full Text Available Cilia are antenna-like extensions of the plasma membrane found in nearly all cell types. In the retina of the eye, photoreceptors develop unique sensory cilia. Not much was known about the mechanisms underlying the formation and function of photoreceptor cilia, largely because of technical limitations and the specific structural and functional modifications that cannot be modeled in vitro. With recent advances in microscopy techniques and molecular and biochemical approaches, we are now beginning to understand the molecular basis of photoreceptor ciliary architecture, ciliary function and its involvement in human diseases. Here, I will discuss the studies that have revealed new knowledge of how photoreceptor cilia regulate their identity and function while coping with high metabolic and trafficking demands associated with processing light signal.

Downstream collecting in ciliary suspension feeders: the catch-up principle

DEFF Research Database (Denmark)

Riisgård, Hans Ulrik; Nielsen, Claus; Larsen, Poul Scheel

2000-01-01

-size retention spectrum, the lower limit depends on spacing between cilia in phase, while the upper end depends on cilia length which may or may not allow particles to enter the ciliary region. On the basis of fluid mechanical considerations and literature descriptions of structure and function of the ciliary......Based on observations of feeding structures and currents in the polychaete Spirorbis tridentatus, the entoproct Loxosoma pectinaricola and the cycliophore Symbion pandora, which all possess compound cilia, it is hypothesized that their capture mechanism is based on the catch-up principle. According...... to this principle, the compound cilia constitute the pump which generates a flow with suspended particles that enters the ciliary region. In this region the same cilia, during their power stroke, catch up with suspended particles and transfer the particles to a food groove, or a mouth cavity. In the particle...

Arf4 is required for Mammalian development but dispensable for ciliary assembly.

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John A Follit

2014-02-01

Full Text Available The primary cilium is a sensory organelle, defects in which cause a wide range of human diseases including retinal degeneration, polycystic kidney disease and birth defects. The sensory functions of cilia require specific receptors to be targeted to the ciliary subdomain of the plasma membrane. Arf4 has been proposed to sort cargo destined for the cilium at the Golgi complex and deemed a key regulator of ciliary protein trafficking. In this work, we show that Arf4 binds to the ciliary targeting sequence (CTS of fibrocystin. Knockdown of Arf4 indicates that it is not absolutely required for trafficking of the fibrocystin CTS to cilia as steady-state CTS levels are unaffected. However, we did observe a delay in delivery of newly synthesized CTS from the Golgi complex to the cilium when Arf4 was reduced. Arf4 mutant mice are embryonic lethal and die at mid-gestation shortly after node formation. Nodal cilia appeared normal and functioned properly to break left-right symmetry in Arf4 mutant embryos. At this stage of development Arf4 expression is highest in the visceral endoderm but we did not detect cilia on these cells. In the visceral endoderm, the lack of Arf4 caused defects in cell structure and apical protein localization. This work suggests that while Arf4 is not required for ciliary assembly, it is important for the efficient transport of fibrocystin to cilia, and also plays critical roles in non-ciliary processes.

A novel ICK mutation causes ciliary disruption and lethal endocrine-cerebro-osteodysplasia syndrome.

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Oud, Machteld M; Bonnard, Carine; Mans, Dorus A; Altunoglu, Umut; Tohari, Sumanty; Ng, Alvin Yu Jin; Eskin, Ascia; Lee, Hane; Rupar, C Anthony; de Wagenaar, Nathalie P; Wu, Ka Man; Lahiry, Piya; Pazour, Gregory J; Nelson, Stanley F; Hegele, Robert A; Roepman, Ronald; Kayserili, Hülya; Venkatesh, Byrappa; Siu, Victoria M; Reversade, Bruno; Arts, Heleen H

2016-01-01

Endocrine-cerebro-osteodysplasia (ECO) syndrome [MIM:612651] caused by a recessive mutation (p.R272Q) in Intestinal cell kinase (ICK) shows significant clinical overlap with ciliary disorders. Similarities are strongest between ECO syndrome, the Majewski and Mohr-Majewski short-rib thoracic dysplasia (SRTD) with polydactyly syndromes, and hydrolethalus syndrome. In this study, we present a novel homozygous ICK mutation in a fetus with ECO syndrome and compare the effect of this mutation with the previously reported ICK variant on ciliogenesis and cilium morphology. Through homozygosity mapping and whole-exome sequencing, we identified a second variant (c.358G > T; p.G120C) in ICK in a Turkish fetus presenting with ECO syndrome. In vitro studies of wild-type and mutant mRFP-ICK (p.G120C and p.R272Q) revealed that, in contrast to the wild-type protein that localizes along the ciliary axoneme and/or is present in the ciliary base, mutant proteins rather enrich in the ciliary tip. In addition, immunocytochemistry revealed a decreased number of cilia in ICK p.R272Q-affected cells. Through identification of a novel ICK mutation, we confirm that disruption of ICK causes ECO syndrome, which clinically overlaps with the spectrum of ciliopathies. Expression of ICK-mutated proteins result in an abnormal ciliary localization compared to wild-type protein. Primary fibroblasts derived from an individual with ECO syndrome display ciliogenesis defects. In aggregate, our findings are consistent with recent reports that show that ICK regulates ciliary biology in vitro and in mice, confirming that ECO syndrome is a severe ciliopathy.

Effect of N-acetylcysteine on the human nasal ciliary activity in vitro

DEFF Research Database (Denmark)

Stafanger, G; Bisgaard, H; Pedersen, M

1987-01-01

N-acetylcysteine (NAC) is widely used as a mucolytic agent, but the clinical and pharmacological effects of NAC are still unclear. It has recently been claimed in animal studies that NAC will stimulate ciliary beating frequency at low concentrations, while inhibiting beating at higher concentrati......N-acetylcysteine (NAC) is widely used as a mucolytic agent, but the clinical and pharmacological effects of NAC are still unclear. It has recently been claimed in animal studies that NAC will stimulate ciliary beating frequency at low concentrations, while inhibiting beating at higher...... concentrations. Using a microphoto-oscillographic method combined with microperfusion technique, we studied the direct effect of NAC on human nasal cilia. NAC caused a direct dose- and time-related decrease in ciliary beating frequency, which was detectable at 2 mg/ml and reached statistically significant levels...

Long-term clearance from small airways in subjects with ciliary dysfunction

OpenAIRE

Hjelte Lena; Falk Rolf; Lindström Maria; Philipson Klas; Svartengren Magnus

2006-01-01

Abstract The objective of this study was to investigate if long-term clearance from small airways is dependent on normal ciliary function. Six young adults with primary ciliary dyskinesia (PCD) inhaled 111 Indium labelled Teflon particles of 4.2 μm geometric and 6.2 μm aerodynamic diameter with an extremely slow inhalation flow, 0.05 L/s. The inhalation method deposits particles mainly in the small conducting airways. Lung retention was measured immediately after inhalation and at four occasi...

Protein Interaction Analysis Provides a Map of the Spatial and Temporal Organization of the Ciliary Gating Zone.

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Takao, Daisuke; Wang, Liang; Boss, Allison; Verhey, Kristen J

2017-08-07

The motility and signaling functions of the primary cilium require a unique protein and lipid composition that is determined by gating mechanisms localized at the base of the cilium. Several protein complexes localize to the gating zone and may regulate ciliary protein composition; however, the mechanisms of ciliary gating and the dynamics of the gating components are largely unknown. Here, we used the BiFC (bimolecular fluorescence complementation) assay and report for the first time on the protein-protein interactions that occur between ciliary gating components and transiting cargoes during ciliary entry. We find that the nucleoporin Nup62 and the C termini of the nephronophthisis (NPHP) proteins NPHP4 and NPHP5 interact with the axoneme-associated kinesin-2 motor KIF17 and thus spatially map to the inner region of the ciliary gating zone. Nup62 and NPHP4 exhibit rapid turnover at the transition zone and thus define dynamic components of the gate. We find that B9D1, AHI1, and the N termini of NPHP4 and NPHP5 interact with the transmembrane protein SSTR3 and thus spatially map to the outer region of the ciliary gating zone. B9D1, AHI1, and NPHP5 exhibit little to no turnover at the transition zone and thus define components of a stable gating structure. These data provide the first comprehensive map of the molecular orientations of gating zone components along the inner-to-outer axis of the ciliary gating zone. These results advance our understanding of the functional roles of gating zone components in regulating ciliary protein composition. Copyright © 2017 Elsevier Ltd. All rights reserved.

Cyanide levels found in infected cystic fibrosis sputum inhibit airway ciliary function.

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Nair, Chandrika; Shoemark, Amelia; Chan, Mario; Ollosson, Sarah; Dixon, Mellissa; Hogg, Claire; Alton, Eric W F W; Davies, Jane C; Williams, Huw D

2014-11-01

We have previously reported cyanide at concentrations of up to 150 μM in the sputum of cystic fibrosis patients infected with Pseudomonas aeruginosa and a negative correlation with lung function. Our aim was to investigate possible mechanisms for this association, focusing on the effect of pathophysiologically relevant cyanide levels on human respiratory cell function. Ciliary beat frequency measurements were performed on nasal brushings and nasal air-liquid interface (ALI) cultures obtained from healthy volunteers and cystic fibrosis patients. Potassium cyanide decreased ciliary beat frequency in healthy nasal brushings (n = 6) after 60 min (150 μM: 47% fall, pcyanide as a key component inhibiting the ciliary beat frequency. If cyanide production similarly impairs mucocilliary clearance in vivo, it could explain the link with increased disease severity observed in cystic fibrosis patients with detectable cyanide in their airway. ©ERS 2014.

Effect of cAMP on short-circuit current in isolated human ciliary body.

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Wu, Ren-yi; Ma, Ning; Hu, Qian-qian

2013-07-01

Cyclic adenosine monophosphate (cAMP) could activate chloride channels in bovine ciliary body and trigger an increase in the ionic current (short-circuit current, Isc) across the ciliary processes in pigs. The purpose of this study was to investigate how cAMP modulates Isc in isolated human ciliary processes and the possible involvement of chloride transport across the tissue in cAMP-induced Isc change. In an Ussing-type chamber system, the Isc changes induced by the cAMP analogue 8-bromo-cAMP and an adenylyl cyclase activator forskolin in isolated human ciliary processes were assessed. The involvement of Cl(-) component in the bath solution was investigated. The effect of Cl(-) channel (10 µmol/L niflumic acid and 1 mmol/L 4,4'-diisothiocyanostilbene-2,2'-disulfonic acid (DIDS)), K(+) channel (10 mmol/L tetraethylammonium chloride (TEA)), or Na(+) channel blockers (1 mmol/L amiloride) on 8-bromo-cAMP-induced Isc change was also studied. Dose-dependently, 8-bromo-cAMP (10 nmol/L-30 µmol/L) or forskolin (10 nmol/L-3 µmol/L) increased Isc across the ciliary processes with an increase in negative potential difference on the non-pigmented epithelium (NPE) side of the tissue. Isc increase induced by 8-bromo-cAMP was more pronounced when the drug was applied on the NPE side than on the pigmented epithelium side. When the tissue was bathed in low Cl(-) solutions, the Isc increase was significantly inhibited. Finally, niflumic acid and DIDS, but not TEA or amiloride, significantly prevented the Isc increase induced by 8-bromo-cAMP. cAMP stimulates stroma-to-aqueous anionic transport in isolated human ciliary processes. Chloride is likely to be among the ions, the transportation of which across the tissue is triggered by cAMP, suggesting the potential role of cAMP in the process of aqueous humor formation in human eyes.

Ciliary body melanoma with optic nerve invasion.

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al-Haddab, S; Hidayat, A; Tabbara, K F

1990-01-01

A case of melanoma of the ciliary body is presented. Initially the patient was diagnosed and treated for uveitis, but following CT scanning and ultrasound a tumour was detected and the eye enucleated. Histopathologically it was found that the tumour had invaded the optic nerve head, apparently via Cloquet's canal. Images PMID:2310725

Bug22 influences cilium morphology and the post-translational modification of ciliary microtubules

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Teresa Mendes Maia

2014-01-01

Cilia and flagella are organelles essential for motility and sensing of environmental stimuli. Depending on the cell type, cilia acquire a defined set of functions and, accordingly, are built with an appropriate length and molecular composition. Several ciliary proteins display a high degree of conservation throughout evolution and mutations in ciliary genes are associated with various diseases such as ciliopathies and infertility. Here, we describe the role of the highly conserved ciliary protein, Bug22, in Drosophila. Previous studies in unicellular organisms have shown that Bug22 is required for proper cilia function, but its exact role in ciliogenesis has not been investigated yet. Null Bug22 mutant flies display cilia-associated phenotypes and nervous system defects. Furthermore, sperm differentiation is blocked at the individualization stage, due to impaired migration of the individualization machinery. Tubulin post-translational modifications (PTMs such as polyglycylation, polyglutamylation or acetylation, are determinants of microtubule (MT functions and stability in centrioles, cilia and neurons. We found defects in the timely incorporation of polyglycylation in sperm axonemal MTs of Bug22 mutants. In addition, we found that depletion of human Bug22 in RPE1 cells resulted in the appearance of longer cilia and reduced axonemal polyglutamylation. Our work identifies Bug22 as a protein that plays a conserved role in the regulation of PTMs of the ciliary axoneme.

Long-term outcome of Tunisian children with primary ciliary ...

African Journals Online (AJOL)

Objective: To study the long-term outcome of PCD in Tunisian children with ciliary ultra-structure defects detected by electron .... The middle lobe was as much affected as the right up- .... distress syndrome (ARDS) after one and four years of.

Bacteriology and treatment of infections in the upper and lower airways in patients with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Alanin, Mikkel Christian

2017-01-01

The respiratory tract is lined with motile cilia that transport respiratory mucus. Primary ciliary dyskinesia (PCD) is a chronic genetic disease caused by mutations in genes responsible for ciliary structure and function. Non-functional airway cilia impair the mucociliary clearance (MCC), causing...

Intraflagellar Transport (IFT) Role in Ciliary Assembly, Resorption and Signalling

DEFF Research Database (Denmark)

Pedersen, Lotte B; Rosenbaum, Joel L

2008-01-01

Cilia and flagella have attracted tremendous attention in recent years as research demonstrated crucial roles for these organelles in coordinating a number of physiologically and developmentally important signaling pathways, including the platelet-derived growth factor receptor (PDGFR) alpha, Sonic...... hedgehog, polycystin, and Wnt pathways. In addition, the realization that defective assembly or function of cilia can cause a plethora of diseases and developmental defects ("ciliopathies") has increased focus on the mechanisms by which these antenna-like, microtubular structures assemble. Ciliogenesis...... mechanisms and functions of IFT. In addition to a general, up-to-date description of IFT, we discuss mechanisms by which proteins are selectively targeted to the ciliary compartment, with special focus on the ciliary transition zone. Finally, we briefly review the role of IFT in cilia-mediated signaling...

Regulation of anterior chamber drainage by bicarbonate-sensitive soluble adenylyl cyclase in the ciliary body.

Science.gov (United States)

Lee, Yong S; Tresguerres, Martin; Hess, Kenneth; Marmorstein, Lihua Y; Levin, Lonny R; Buck, Jochen; Marmorstein, Alan D

2011-12-02

Glaucoma is a leading cause of blindness affecting as many as 2.2 million Americans. All current glaucoma treatment strategies aim to reduce intraocular pressure (IOP). IOP results from the resistance to drainage of aqueous humor (AH) produced by the ciliary body in a process requiring bicarbonate. Once secreted into the anterior chamber, AH drains from the eye via two pathways: uveoscleral and pressure-dependent or conventional outflow (C(t)). Modulation of "inflow" and "outflow" pathways is thought to occur via distinct, local mechanisms. Mice deficient in the bicarbonate channel bestrophin-2 (Best2), however, exhibit a lower IOP despite an increase in AH production. Best2 is expressed uniquely in nonpigmented ciliary epithelial (NPE) cells providing evidence for a bicarbonate-dependent communicative pathway linking inflow and outflow. Here, we show that bicarbonate-sensitive soluble adenylyl cyclase (sAC) is highly expressed in the ciliary body in NPE cells, but appears to be absent from drainage tissues. Pharmacologic inhibition of sAC in mice causes a significant increase in IOP due to a decrease in C(t) with no effect on inflow. In mice deficient in sAC IOP is elevated, and C(t) is decreased relative to wild-type mice. Pharmacologic inhibition of sAC did not alter IOP or C(t) in sAC-deficient mice. Based on these data we propose that the ciliary body can regulate C(t) and that sAC serves as a critical sensor of bicarbonate in the ciliary body regulating the secretion of substances into the AH that govern outflow facility independent of pressure.

LRRC6 mutation causes primary ciliary dyskinesia with dynein arm defects.

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Amjad Horani

Full Text Available Despite recent progress in defining the ciliome, the genetic basis for many cases of primary ciliary dyskinesia (PCD remains elusive. We evaluated five children from two unrelated, consanguineous Palestinian families who had PCD with typical clinical features, reduced nasal nitric oxide concentrations, and absent dynein arms. Linkage analyses revealed a single common homozygous region on chromosome 8 and one candidate was conserved in organisms with motile cilia. Sequencing revealed a single novel mutation in LRRC6 (Leucine-rich repeat containing protein 6 that fit the model of autosomal recessive genetic transmission, leading to a change of a highly conserved amino acid from aspartic acid to histidine (Asp146His. LRRC6 was localized to the cytoplasm and was up-regulated during ciliogenesis in human airway epithelial cells in a Foxj1-dependent fashion. Nasal epithelial cells isolated from affected individuals and shRNA-mediated silencing in human airway epithelial cells, showed reduced LRRC6 expression, absent dynein arms, and slowed cilia beat frequency. Dynein arm proteins were either absent or mislocalized to the cytoplasm in airway epithelial cells from a primary ciliary dyskinesia subject. These findings suggest that LRRC6 plays a role in dynein arm assembly or trafficking and when mutated leads to primary ciliary dyskinesia with laterality defects.

Alteration of sodium, potassium-adenosine triphosphatase activity in rabbit ciliary processes by cyclic adenosine monophosphate-dependent protein kinase

International Nuclear Information System (INIS)

Delamere, N.A.; Socci, R.R.; King, K.L.

1990-01-01

The response of sodium, potassium-adenosine triphosphatase (Na,K-ATPase) to cyclic adenosine monophosphate (cAMP)-dependent protein kinase was examined in membranes obtained from rabbit iris-ciliary body. In the presence of the protein kinase together with 10(-5) M cAMP, Na,K-ATPase activity was reduced. No change in Na,K-ATPase activity was detected in response to the protein kinase without added cAMP. Likewise cAMP alone did not alter Na,K-ATPase activity. Reduction of Na,K-ATPase activity was also observed in the presence of the cAMP-dependent protein kinase catalytic subunit. The response of the enzyme to the kinase catalytic subunit was also examined in membranes obtained from rabbit ciliary processes. In the presence of 8 micrograms/ml of the catalytic subunit, ciliary process Na,K-ATPase activity was reduced by more than 50%. To examine whether other ATPases were suppressed by the protein kinase, calcium-stimulated ATPase activity was examined; its activity was stimulated by the catalytic subunit. To test whether the response of the ciliary process Na,K-ATPase is unique, experiments were also performed using membrane preparations from rabbit lens epithelium or rabbit kidney; the catalytic subunit significantly reduced the activity of Na,K-ATPase from the kidney but not the lens. These Na,K-ATPase studies suggest that in the iris-ciliary body, cAMP may alter sodium pump activity. In parallel 86Rb uptake studies, we observed that ouabain-inhibitable potassium uptake by intact pieces of iris-ciliary body was reduced by exogenous dibutryl cAMP or by forskolin

Ciliary neurotrophic factor is an endogenous pyrogen.

OpenAIRE

Shapiro, L; Zhang, X X; Rupp, R G; Wolff, S M; Dinarello, C A

1993-01-01

Fever is initiated by the action of polypeptide cytokines called endogenous pyrogens, which are produced by the host during inflammation, trauma, or infection and which elevate the thermoregulatory set point in the hypothalamus. Ciliary neurotrophic factor (CNTF) supports the differentiation and survival of central and peripheral neurons. We describe the activity of CNTF as intrinsically pyrogenic in the rabbit. CNTF induced a monophasic fever which rose rapidly (within the first 12 min) foll...

Long-term clearance from small airways in subjects with ciliary dysfunction

Directory of Open Access Journals (Sweden)

Hjelte Lena

2006-05-01

Full Text Available Abstract The objective of this study was to investigate if long-term clearance from small airways is dependent on normal ciliary function. Six young adults with primary ciliary dyskinesia (PCD inhaled 111 Indium labelled Teflon particles of 4.2 μm geometric and 6.2 μm aerodynamic diameter with an extremely slow inhalation flow, 0.05 L/s. The inhalation method deposits particles mainly in the small conducting airways. Lung retention was measured immediately after inhalation and at four occasions up to 21 days after inhalation. Results were compared with data from ten healthy controls. For additional comparison three of the PCD subjects also inhaled the test particles with normal inhalation flow, 0.5 L/s, providing a more central deposition. The lung retention at 24 h in % of lung deposition (Ret24 was higher (p 24 with slow inhalation flow was 73.9 ± 1.9 % compared to 68.9 ± 7.5 % with normal inhalation flow in the three PCD subjects exposed twice. During day 7–21 the three PCD subjects exposed twice cleared 9 % with normal flow, probably representing predominantly alveolar clearance, compared to 19 % with slow inhalation flow, probably representing mainly small airway clearance. This study shows that despite ciliary dysfunction, clearance continues in the small airways beyond 24 h. There are apparently additional clearance mechanisms present in the small airways.

Loss and gain of cone types in vertebrate ciliary photoreceptor evolution.

Science.gov (United States)

Musser, Jacob M; Arendt, Detlev

2017-11-01

Ciliary photoreceptors are a diverse cell type family that comprises the rods and cones of the retina and other related cell types such as pineal photoreceptors. Ciliary photoreceptor evolution has been dynamic during vertebrate evolution with numerous gains and losses of opsin and phototransduction genes, and changes in their expression. For example, early mammals lost all but two cone opsins, indicating loss of cone receptor types in response to nocturnal lifestyle. Our review focuses on the comparison of specifying transcription factors and cell type-specific transcriptome data in vertebrate retinae to build and test hypotheses on ciliary photoreceptor evolution. Regarding cones, recent data reveal that a combination of factors specific for long-wavelength sensitive opsin (Lws)- cones in non-mammalian vertebrates (Thrb and Rxrg) is found across all differentiating cone photoreceptors in mice. This suggests that mammalian ancestors lost all but one ancestral cone type, the Lws-cone. We test this hypothesis by a correlation analysis of cone transcriptomes in mouse and chick, and find that, indeed, transcriptomes of all mouse cones are most highly correlated to avian Lws-cones. These findings underscore the importance of specifying transcription factors in tracking cell type evolution, and shed new light on the mechanisms of cell type loss and gain in retina evolution. Copyright © 2017 The Authors. Published by Elsevier Inc. All rights reserved.

Particulate matter in cigarette smoke increases ciliary axoneme beating through mechanical stimulation.

Science.gov (United States)

Navarrette, Chelsea R; Sisson, Joseph H; Nance, Elizabeth; Allen-Gipson, Diane; Hanes, Justin; Wyatt, Todd A

2012-06-01

The lung's ability to trap and clear foreign particles via the mucociliary elevator is an important mechanism for protecting the lung against respirable irritants and microorganisms. Although cigarette smoke (CS) exposure and particulate inhalation are known to alter mucociliary clearance, little is known about how CS and nanoparticles (NPs) modify cilia beating at the cytoskeletal infrastructure, or axonemal, level. We used a cell-free model to introduce cigarette smoke extract (CSE) and NPs with variant size and surface chemistry to isolated axonemes and measured changes in ciliary motility. We hypothesized that CSE would alter cilia beating and that alterations in ciliary beat frequency (CBF) due to particulate matter would be size- and surface chemistry-dependent. Demembranated axonemes were isolated from ciliated bovine tracheas and exposed to adenosine triphosphate (ATP) to initiate motility. CBF was measured in response to 5% CSE, CSE filtrate, and carboxyl-modified (COOH), sulphate (SO(4))-modified (sulfonated), or PEG-coated polystyrene (PS) latex NPs ranging in size from 40 nm to 500 nm. CSE concentrations as low as 5% resulted in rapid, significant stimulation of CBF (pIntroduction of sulphate-modified PS beads ~300 nm in diameter resulted in a similar increase in CBF above baseline ATP levels. Uncharged, PEG-coated beads had no effect on CBF regardless of size. Similarly, COOH-coated particles less than 200 nm in diameter did not alter ciliary motility. However, COOH-coated PS particles larger than 300 nm increased CBF significantly and increased the number of motile points. These data show that NPs, including those found in CSE, mechanically stimulate axonemes in a size- and surface chemistry-dependent manner. Alterations in ciliary motility due to physicochemical properties of NPs may be important for inhalational lung injury and efficient drug delivery of respirable particles.

Asymmetric wavefront aberrations and pupillary shapes induced by electrical stimulation of ciliary nerve in cats measured with compact wavefront aberrometer.

Directory of Open Access Journals (Sweden)

Suguru Miyagawa

Full Text Available To investigate the changes in the wavefront aberrations and pupillary shape in response to electrical stimulation of the branches of the ciliary nerves in cats. Seven eyes of seven cats were studied under general anesthesia. Trains of monophasic pulses (current, 0.1 to 1.0 mA; duration, 0.5 ms/phase; frequency, 5 to 40 Hz were applied to the lateral or medial branch of the short ciliary nerve near the posterior pole of the eye. A pair of electrodes was hooked onto one or both branch of the short ciliary nerve. The electrodes were placed about 5 mm from the scleral surface. The wavefront aberrations were recorded continuously for 2 seconds before, 8 seconds during, and for 20 seconds after the electrical stimulation. The pupillary images were simultaneously recorded during the stimulation period. Both the wavefront aberrations and the pupillary images were obtained 10 times/sec with a custom-built wavefront aberrometer. The maximum accommodative amplitude was 1.19 diopters (D produced by electrical stimulation of the short ciliary nerves. The latency of the accommodative changes was very short, and the accommodative level gradually increased up to 4 seconds and reached a plateau. When only one branch of the ciliary nerve was stimulated, the pupil dilated asymmetrically, and the oblique astigmatism and one of the asymmetrical wavefront terms was also altered. Our results showed that the wavefront aberrations and pupillary dilations can be measured simultaneously and serially with a compact wavefront aberrometer. The asymmetric pupil dilation and asymmetric changes of the wavefront aberrations suggest that each branch of the ciliary nerve innervates specific segments of the ciliary muscle and dilator muscle of the pupil.

Recent invasion of buffel grass (Cenchrus ciliaris of a natural protected area from the southern Sonoran Desert Invasión reciente de zacate buffel (Cenchrus ciliaris en un área natural protegida del desierto sonorense

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Erick De la Barrera

2008-12-01

Full Text Available The Centro Ecológico de Sonora is a natural protected area where the natural vegetation remained undisturbed at least until 1997. Since then, Cenchrus ciliaris has become a prominent element of the vegetation because of disturbance. Climate, soil properties, population structure and biological activity for C. ciliaris were studied to gain understanding of the ecological mechanisms that favored the invasion by this exotic grass. Mean air temperature and annual rainfall were 24.8°C and 302 mm. The soil was a loamy-sand that was poor in most nutrients, but particularly rich in phosphorus. Pennisetum ciliare was the most abundant species at the Centro Ecológico, representing over one third of total plant ground cover. Basal area for individual plants ranged from less than 1 cm² to almost 1 m². Living leaves per plant increased with precipitation, peaking at 199 leaves in March 2005, and no living leaves were found after 103 days without rain. The environmental conditions prevalent at Centro Ecológico are very favorable for C. ciliaris, whose establishment was apparently triggered by a major disturbance caused by the development of housing projects.El Centro Ecológico de Sonora es un área natural protegida donde la vegetación autóctona permaneció sin disturbios por lo menos hasta 1997. Desde entonces, Cenchrus ciliaris se ha convertido en un elemento prominente de la vegetación. Se estudiaron el clima, las propiedades del suelo, la estructura de la población y la actividad biológica de C. ciliaris, como una aproximación al entendimiento de los mecanismos ecológicos que favorecieron la invasión por este pasto exótico. La temperatura media del aire y la precipitación anual fueron de 24.8 °C y 302 mm. El suelo fue una arena limosa pobre en minerales, pero particularmente rica en fósforo. Cenchrus ciliaris fue la especie herbácea más abundante en el Centro Ecológico, representando más de un tercio de la cobertura vegetal. El �

Simultaneous sinus and lung infections in patients with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Alanin, Mikkel Christian; Johansen, Helle Krogh; Aanaes, Kasper

2015-01-01

Conclusion: The sinuses should be considered as a bacterial reservoir and a target for surgery and antibiotic treatment in patients with primary ciliary dyskinesia (PCD). The observed decrease in serum precipitating antibodies (precipitins) against Pseudomonas aeruginosa may indicate a beneficial...

Paramecium swimming and ciliary beating patterns: a study on four RNA interference mutations.

Science.gov (United States)

Funfak, Anette; Fisch, Cathy; Abdel Motaal, Hatem T; Diener, Julien; Combettes, Laurent; Baroud, Charles N; Dupuis-Williams, Pascale

2015-01-01

Paramecium cells swim and feed by beating their thousands of cilia in coordinated patterns. The organization of these patterns and its relationship with cell motility has been the subject of a large body of work, particularly as a model for ciliary beating in human organs where similar organization is seen. However the rapid motion of the cells makes quantitative measurements very challenging. Here we provide detailed measurements of the swimming of Paramecium cells from high-speed video at high magnification, as they move in microfluidic channels. An image analysis protocol allows us to decouple the cell movement from the motion of the cilia, thus allowing us to measure the ciliary beat frequency (CBF) and the spatio-temporal organization into metachronal waves along the cell periphery. Two distinct values of the CBF appear at different regions of the cell: most of the cilia beat in the range of 15 to 45 Hz, while the cilia in the peristomal region beat at almost double the frequency. The body and peristomal CBF display a nearly linear relation with the swimming velocity. Moreover the measurements do not display a measurable correlation between the swimming velocity and the metachronal wave velocity on the cell periphery. These measurements are repeated for four RNAi silenced mutants, where proteins specific to the cilia or to their connection to the cell base are depleted. We find that the mutants whose ciliary structure is affected display similar swimming to the control cells albeit with a reduced efficiency, while the mutations that affect the cilia's anchoring to the cell lead to strongly reduced ability to swim. This reduction in motility can be related to a loss of coordination between the ciliary beating in different parts of the cell.

Surgical treatment of iris and ciliary body melanoma: follow-up of a 25-year series of patients

DEFF Research Database (Denmark)

Klauber, Stefan; Jensen, Peter K; Prause, Jan U

2010-01-01

Purpose: To evaluate outcome of surgical resection of iris and irido-ciliary melanomas. Method: Retrospective analysis of all cases treated in Denmark 1975-1999 with clinical follow-up in 2002 and death certificate analysis in 2008. A quality of life questionnaire was completed at follow-up. Resu......Purpose: To evaluate outcome of surgical resection of iris and irido-ciliary melanomas. Method: Retrospective analysis of all cases treated in Denmark 1975-1999 with clinical follow-up in 2002 and death certificate analysis in 2008. A quality of life questionnaire was completed at follow...... time. However, none had changed job as a consequence of the surgical treatment. Only two patients were emotionally affected by the diagnosis of iris melanoma. Conclusion: Resection of small iris and irido-ciliary melanomas is a safe and efficient procedure, provided that strict diagnostic and surgical...

Regulation of protein phosphorylation of the intermediate-sized filament vimentin in the ciliary epithelium of the mammalian eye

International Nuclear Information System (INIS)

Coca-Prados, M.

1985-01-01

The intermediate-sized filaments of vimentin-type (Mr = 57,000) have been identified biochemically and immunochemically as a major cytoskeleton component in the ciliary epithelium of the mammalian eye. When human or rabbit ciliary processes, or cultured ciliary epithelial-derived cells were incubated in serum-free medium containing [ 32 P]orthophosphate and any of the following agents: 1) beta-adrenergic agonists (isoproterenol or epinephrine), 2) direct activators of adenylate cyclase (cholera toxin or forskolin), 3) analogs of cyclic AMP (8-Br-cAMP), or 4) prostaglandin E1, the phosphorylation of vimentin was significantly enhanced. The maximal enhancement ranged, in vivo and in vitro, from about 3-fold in human to 5-fold in rabbit, with either 1 mM 8-Br-cAMP or 0.1 microM forskolin. Indirect immunofluorescence microscopy using a monoclonal antibody, anti-vimentin, allowed the localization of vimentin filaments in cultured ciliary epithelial cells. Treatment of these cells in culture with the catecholamine hormone, isoproterenol (1 microM), resulted in a profound reorganization of vimentin filaments. This may be correlated with the enhanced levels of phosphorylated vimentin observed upon increasing cellular cyclic AMP

Methods for Studying Ciliary-Mediated Chemoresponse in Paramecium.

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Valentine, Megan Smith; Van Houten, Judith L

2016-01-01

Paramecium is a useful model organism for the study of ciliary-mediated chemical sensing and response. Here we describe ways to take advantage of Paramecium to study chemoresponse.Unicellular organisms like the ciliated protozoan Paramecium sense and respond to chemicals in their environment (Van Houten, Ann Rev Physiol 54:639-663, 1992; Van Houten, Trends Neurosci 17:62-71, 1994). A thousand or more cilia that cover Paramecium cells serve as antennae for chemical signals, similar to ciliary function in a large variety of metazoan cell types that have primary or motile cilia (Berbari et al., Curr Biol 19(13):R526-R535, 2009; Singla V, Reiter J, Science 313:629-633, 2006). The Paramecium cilia also produce the motor output of the detection of chemical cues by controlling swimming behavior. Therefore, in Paramecium the cilia serve multiple roles of detection and response.We present this chapter in three sections to describe the methods for (1) assaying populations of cells for their behavioral responses to chemicals (attraction and repulsion), (2) characterization of the chemoreceptors and associated channels of the cilia using proteomics and binding assays, and (3) electrophysiological analysis of individual cells' responses to chemicals. These methods are applied to wild type cells, mutants, transformed cells that express tagged proteins, and cells depleted of gene products by RNA Interference (RNAi).

Longitudinal study of lung function in a cohort of primary ciliary dyskinesia

DEFF Research Database (Denmark)

Ellerman, A; Bisgaard, H

1997-01-01

Patients with primary ciliary dyskinesia (PCD) have pronounced stasis of their respiratory secretions and therefore recurrent lower airway infections, which raises concerns for the development of lung function. Twenty four patients with PCD have been studied prospectively with a standardized regime...... patients entering as children (forced vital capacity (FVC) 70 versus 85% predicted; forced expiratory volume in one second (FEV1) 59 versus 72% pred). The lung damage did not relate to the type of ciliary dyskinesia. During the subsequent surveillance of the groups for a median of 14 and 7 yrs...... in our clinic for 2-16 yrs with clinic visits, including spirometry 2-4 times per year, daily physiotherapy and monthly sputum cultures with subsequent specific antibiotic treatment. Lung function was significantly lower in the 12 PCD patients entering the cohort as adults when compared to the PCD...

Functional short- and long-term effects of nasal CPAP with and without humidification on the ciliary function of the nasal respiratory epithelium.

Science.gov (United States)

Sommer, J Ulrich; Kraus, Marius; Birk, Richard; Schultz, Johannes D; Hörmann, Karl; Stuck, Boris A

2014-03-01

Continuous positive airway pressure (CPAP) is the gold standard in the treatment of obstructive sleep apnea (OSA), but its impact on ciliary function is unclear to date. Furthermore, CPAP is associated with numerous side effects related to the nose and upper airway. Humidified CPAP is used to relieve these symptoms, but again, little is known regarding its effect on ciliary function of the nasal respiratory epithelium. In this prospective, randomized, crossover trial, 31 patients with OSA (AHI >15/h) were randomized to two treatment arms: nasal continuous positive airway pressure (nCPAP) with humidification or nCPAP without humidification for one night in each modality to assess short-term effects of ciliary beat frequency (CBF) and mucus transport time (MTT) and consecutively for 8 weeks in each modality to assess long-term effects in a crossover fashion. The baseline CBF was 4.8 ± 0.6 Hz, and baseline MTT was 540 ± 221 s. After one night of CPAP with and without humidification, ciliary function increased moderately yet with statistical significance (p humidification did not differ statistically significant. Regarding long-term effects of CPAP, a statistically significant increase in ciliary function above the baseline level and above the short-term level was shown without humidification (7.2 ± 0.4 Hz; 402 ± 176 s; p humidification (9.3 ± 0.7 Hz; 313 ± 95 s; p humidification, nCPAP has moderate effects on short-term ciliary function of the nasal respiratory epithelium. However, a significant increase in ciliary function-both in terms of an increased CBF and a decreased MTT-was detected after long-term use. The effect was more pronounced when humidification was used during nCPAP.

Gene Expression and Functional Annotation of the Human Ciliary Body Epithelia

NARCIS (Netherlands)

Janssen, Sarah F.; Gorgels, Theo G. M. F.; Bossers, Koen; ten Brink, Jacoline B.; Essing, Anke H. W.; Nagtegaal, Martijn; van der Spek, Peter J.; Jansonius, Nomdo M.; Bergen, Arthur A. B.

2012-01-01

Purpose: The ciliary body (CB) of the human eye consists of the non-pigmented (NPE) and pigmented (PE) neuro-epithelia. We investigated the gene expression of NPE and PE, to shed light on the molecular mechanisms underlying the most important functions of the CB. We also developed molecular

Automatic analysis of ciliary beat frequency using optical flow

Science.gov (United States)

Figl, Michael; Lechner, Manuel; Werther, Tobias; Horak, Fritz; Hummel, Johann; Birkfellner, Wolfgang

2012-02-01

Ciliary beat frequency (CBF) can be a useful parameter for diagnosis of several diseases, as e.g. primary ciliary dyskinesia. (PCD). CBF computation is usually done using manual evaluation of high speed video sequences, a tedious, observer dependent, and not very accurate procedure. We used the OpenCV's pyramidal implementation of the Lukas-Kanade algorithm for optical flow computation and applied this to certain objects to follow the movements. The objects were chosen by their contrast applying the corner detection by Shi and Tomasi. Discrimination between background/noise and cilia by a frequency histogram allowed to compute the CBF. Frequency analysis was done using the Fourier transform in matlab. The correct number of Fourier summands was found by the slope in an approximation curve. The method showed to be usable to distinguish between healthy and diseased samples. However there remain difficulties in automatically identifying the cilia, and also in finding enough high contrast cilia in the image. Furthermore the some of the higher contrast cilia are lost (and sometimes found) by the method, an easy way to distinguish the correct sub-path of a point's path have yet to be found in the case where the slope methods doesn't work.

Species variation in biology and physiology of the ciliary epithelium: similarities and differences.

Science.gov (United States)

Do, Chi Wai; Civan, Mortimer M

2009-04-01

Glaucoma is a leading cause of irreversible blindness worldwide. Lowering intraocular pressure (IOP) is the only strategy documented to delay the appearance and retard the progression of vision loss. One major approach for lowering IOP is to slow the rate of aqueous humor formation by the ciliary epithelium. As discussed in the present review, the transport basis for this secretion is largely understood. However, several substantive issues are yet to be resolved, including the integrated regulation of secretion, the functional topography of the ciliary epithelium, and the degree and significance of species variation in aqueous humor inflow. This review discusses species differences in net secretion, particularly of Cl(-) and HCO(3)(-) secretion. Identifying animal models most accurately mimicking aqueous humor formation in the human will facilitate development of future novel initiatives to lower IOP.

Bug22p, a conserved centrosomal/ciliary protein also present in higher plants, is required for an effective ciliary stroke in Paramecium.

Science.gov (United States)

Laligné, C; Klotz, C; de Loubresse, N Garreau; Lemullois, M; Hori, M; Laurent, F X; Papon, J F; Louis, B; Cohen, J; Koll, F

2010-04-01

Centrioles, cilia, and flagella are ancestral conserved organelles of eukaryotic cells. Among the proteins identified in the proteomics of ciliary proteins in Paramecium, we focus here on a protein, Bug22p, previously detected by cilia and basal-body high-throughput studies but never analyzed per se. Remarkably, this protein is also present in plants, which lack centrioles and cilia. Bug22p sequence alignments revealed consensus positions that distinguish species with centrioles/cilia from plants. In Paramecium, antibody and green fluorescent protein (GFP) fusion labeling localized Bug22p in basal bodies and cilia, and electron microscopy immunolabeling refined the localization to the terminal plate of the basal bodies, the transition zone, and spots along the axoneme, preferentially between the membrane and the microtubules. RNA interference (RNAi) depletion of Bug22p provoked a strong decrease in swimming speed, followed by cell death after a few days. High-speed video microscopy and morphological analysis of Bug22p-depleted cells showed that the protein plays an important role in the efficiency of ciliary movement by participating in the stroke shape and rigidity of cilia. The defects in cell swimming and growth provoked by RNAi can be complemented by expression of human Bug22p. This is the first reported case of complementation by a human gene in a ciliate.

Recombination within the apospory specific genomic region leads to the uncoupling of apomixis components in Cenchrus ciliaris.

Science.gov (United States)

Conner, Joann A; Gunawan, Gunawati; Ozias-Akins, Peggy

2013-07-01

Apomixis enables the clonal propagation of maternal genotypes through seed. If apomixis could be harnessed via genetic engineering or introgression, it would have a major economic impact for agricultural crops. In the grass species Pennisetum squamulatum and Cenchrus ciliaris (syn. P. ciliare), apomixis is controlled by a single dominant "locus", the apospory-specific genomic region (ASGR). For P. squamulatum, 18 published sequenced characterized amplified region (SCAR) markers have been identified which always co-segregate with apospory. Six of these markers are conserved SCARs in the closely related species, C. ciliaris and co-segregate with the trait. A screen of progeny from a cross of sexual × apomictic C. ciliaris genotypes identified a plant, A8, retaining two of the six ASGR-linked SCAR markers. Additional and newly identified ASGR-linked markers were generated to help identify the extent of recombination within the ASGR. Based on analysis of missing markers, the A8 recombinant plant has lost a significant portion of the ASGR but continues to form aposporous embryo sacs. Seedlings produced from aposporous embryo sacs are 6× in ploidy level and hence the A8 recombinant does not express parthenogenesis. The recombinant A8 plant represents a step forward in reducing the complexity of the ASGR locus to determine the factor(s) required for aposporous embryo sac formation and documents the separation of expression of the two components of apomixis in C. ciliaris.

HEATR2 plays a conserved role in assembly of the ciliary motile apparatus.

Directory of Open Access Journals (Sweden)

Christine P Diggle

2014-09-01

Full Text Available Cilia are highly conserved microtubule-based structures that perform a variety of sensory and motility functions during development and adult homeostasis. In humans, defects specifically affecting motile cilia lead to chronic airway infections, infertility and laterality defects in the genetically heterogeneous disorder Primary Ciliary Dyskinesia (PCD. Using the comparatively simple Drosophila system, in which mechanosensory neurons possess modified motile cilia, we employed a recently elucidated cilia transcriptional RFX-FOX code to identify novel PCD candidate genes. Here, we report characterization of CG31320/HEATR2, which plays a conserved critical role in forming the axonemal dynein arms required for ciliary motility in both flies and humans. Inner and outer arm dyneins are absent from axonemes of CG31320 mutant flies and from PCD individuals with a novel splice-acceptor HEATR2 mutation. Functional conservation of closely arranged RFX-FOX binding sites upstream of HEATR2 orthologues may drive higher cytoplasmic expression of HEATR2 during early motile ciliogenesis. Immunoprecipitation reveals HEATR2 interacts with DNAI2, but not HSP70 or HSP90, distinguishing it from the client/chaperone functions described for other cytoplasmic proteins required for dynein arm assembly such as DNAAF1-4. These data implicate CG31320/HEATR2 in a growing intracellular pre-assembly and transport network that is necessary to deliver functional dynein machinery to the ciliary compartment for integration into the motile axoneme.

Comparative responses of the Savanna grasses Cenchrus ciliaris and Themeda triandra to defoliation.

Science.gov (United States)

Hodgkinson, K C; Ludlow, M M; Mott, J J; Baruch, Z

1989-04-01

Two perennial tussock grasses of savannas were compared in a glasshouse study to determine why they differed in their ability to withstand frequent, heavy grazing; Cenchrus ciliaris is tolerant and Themeda triandra is intolerant of heavy grazing. Frequent defoliation at weekly intervals for six weeks reduced shoot biomass production over a subsequent 42 day regrowth period compared with previously undefoliated plants (infrequent) in T. triandra, but not in C. ciliaris. Leaf area of T. triandra expanded rapidly following defoliation but high initial relative growth rates of shoots were not sustained after 14 days of regrowth because of reducing light utilising efficiency of leaves. Frequently defoliated plants were slower in rate of leaf area expansion and this was associated with reduced photosynthetic capacity of newly formed leaves, lower allocation of photosynthate to leaves but not lower tiller numbers. T. triandra appears well adapted to a regime where defoliation is sufficiently infrequent to allow carbon to be fixed to replace that used in initial leaf area expansion. In contrast, C. ciliaris is better adapted to frequent defoliation than is T. triandra, because horizontally orientated nodal tillers are produced below the defoliation level. This morphological adaptation resulted in a 10-fold higher leaf area remaining after defoliation compared with similarly defoliated T. triandra, which together with the maintenance of moderate levels of light utilising efficiency, contributed to the higher leaf area and shoot weight throughout the regrowth period.

Ventilation inhomogeneity in children with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Green, Kent; Buchvald, Frederik F; Marthin, June Kehlet

2012-01-01

The lung clearance index (LCI) derived from the multiple breath inert gas washout (MBW) test reflects global ventilation distribution inhomogeneity. It is more sensitive than forced expiratory volume in 1 s (FEV(1)) for detecting abnormal airway function and correlates closely with structural lung...... damage in children with cystic fibrosis, which shares features with primary ciliary dyskinesia (PCD). Normalised phase III slope indices S(cond) and S(acin) reflect function of the small conducting and acinar airways, respectively. The involvement of the peripheral airways assessed by MBW tests has...

Diagnosis of primary ciliary dyskinesia: potential options for resource-limited countries

Directory of Open Access Journals (Sweden)

Nisreen Rumman

2017-01-01

Full Text Available Primary ciliary dyskinesia is a genetic disease of ciliary function leading to chronic upper and lower respiratory tract symptoms. The diagnosis is frequently overlooked because the symptoms are nonspecific and the knowledge about the disease in the primary care setting is poor. Additionally, none of the available tests is accurate enough to be used in isolation. These tests are expensive, and need sophisticated equipment and expertise to analyse and interpret results; diagnosis is therefore only available at highly specialised centres. The diagnosis is particularly challenging in countries with limited resources due to the lack of such costly equipment and expertise. In this review, we discuss the importance of early and accurate diagnosis especially for countries where the disease is clinically prevalent but diagnostic tests are lacking. We review the diagnostic tests available in specialised centres (nasal nitric oxide, high-speed video microscopy, transmission electron microscopy, immunofluorescence and genetics. We then consider modifications that might be considered in less well-resourced countries whilst maintaining acceptable accuracy.

ZMYND10 is mutated in primary ciliary dyskinesia and interacts with LRRC6

DEFF Research Database (Denmark)

Zariwala, Maimoona A; Gee, Heon Yung; Kurkowiak, Małgorzata

2013-01-01

Defects of motile cilia cause primary ciliary dyskinesia (PCD), characterized by recurrent respiratory infections and male infertility. Using whole-exome resequencing and high-throughput mutation analysis, we identified recessive biallelic mutations in ZMYND10 in 14 families and mutations in the ...

Mutations in DZIP1L, which encodes a ciliary transition zone protein, cause autosomal recessive polycystic kidney disease

Science.gov (United States)

Lu, Hao; Galeano, Maria C. Rondón; Ott, Elisabeth; Kaeslin, Geraldine; Kausalya, P. Jaya; Kramer, Carina; Ortiz-Brüchle, Nadina; Hilger, Nadescha; Metzis, Vicki; Hiersche, Milan; Tay, Shang Yew; Tunningley, Robert; Vij, Shubha; Courtney, Andrew D.; Whittle, Belinda; Wühl, Elke; Vester, Udo; Hartleben, Björn; Neuber, Steffen; Frank, Valeska; Little, Melissa H.; Epting, Daniel; Papathanasiou, Peter; Perkins, Andrew C.; Wright, Graham D.; Hunziker, Walter; Gee, Heon Yung; Otto, Edgar A.; Zerres, Klaus; Hildebrandt, Friedhelm; Roy, Sudipto; Wicking, Carol; Bergmann, Carsten

2017-01-01

Autosomal recessive polycystic kidney disease (ARPKD), usually considered to be a genetically homogeneous disease caused by mutations in PKHD1, has been associated with ciliary dysfunction. Here, we describe mutations in the DAZ interacting protein 1-like (DZIP1L) gene in patients with ARPKD, findings we have further validated by loss-of-function studies in mice and zebrafish. DZIP1L localizes to centrioles and at the distal end of basal bodies, and interacts with septin2, a protein implicated in maintenance of the periciliary diffusion barrier at the ciliary transition zone. Consistent with a defect in the diffusion barrier, we found that the ciliary membrane translocation of the PKD proteins, polycystin-1 and −2, is compromised in DZIP1L mutant cells. Together, these data provide the first conclusive evidence that ARPKD is not a homogeneous disorder, and establishes DZIP1L as a second gene involved in its pathogenesis. PMID:28530676

Mutations in DZIP1L, which encodes a ciliary-transition-zone protein, cause autosomal recessive polycystic kidney disease.

Science.gov (United States)

Lu, Hao; Galeano, Maria C Rondón; Ott, Elisabeth; Kaeslin, Geraldine; Kausalya, P Jaya; Kramer, Carina; Ortiz-Brüchle, Nadina; Hilger, Nadescha; Metzis, Vicki; Hiersche, Milan; Tay, Shang Yew; Tunningley, Robert; Vij, Shubha; Courtney, Andrew D; Whittle, Belinda; Wühl, Elke; Vester, Udo; Hartleben, Björn; Neuber, Steffen; Frank, Valeska; Little, Melissa H; Epting, Daniel; Papathanasiou, Peter; Perkins, Andrew C; Wright, Graham D; Hunziker, Walter; Gee, Heon Yung; Otto, Edgar A; Zerres, Klaus; Hildebrandt, Friedhelm; Roy, Sudipto; Wicking, Carol; Bergmann, Carsten

2017-07-01

Autosomal recessive polycystic kidney disease (ARPKD), usually considered to be a genetically homogeneous disease caused by mutations in PKHD1, has been associated with ciliary dysfunction. Here, we describe mutations in DZIP1L, which encodes DAZ interacting protein 1-like, in patients with ARPKD. We further validated these findings through loss-of-function studies in mice and zebrafish. DZIP1L localizes to centrioles and to the distal ends of basal bodies, and interacts with septin2, a protein implicated in maintenance of the periciliary diffusion barrier at the ciliary transition zone. In agreement with a defect in the diffusion barrier, we found that the ciliary-membrane translocation of the PKD proteins polycystin-1 and polycystin-2 is compromised in DZIP1L-mutant cells. Together, these data provide what is, to our knowledge, the first conclusive evidence that ARPKD is not a homogeneous disorder and further establish DZIP1L as a second gene involved in ARPKD pathogenesis.

Target recognition and synapse formation by ciliary-ganglion neurons in tissue culture

NARCIS (Netherlands)

Stevens, W.F.; Slaaf, D.W.; Hooisma, J.; Magchielse, T.; Meeter, E.

1978-01-01

A less complicated source of neurons suitable for this type of studies is the parasympathetic ciliary ganglion. In the pigeon and in the chick this ganglion is known to contain only two classes of neurons, both of which are cholinoceptive and cholinergic and that innervate the muscle fibres of the

Cardiopulmonary Exercise Testing in Fontan Patients With and Without Isomerism (Heterotaxy) as Compared to Patients With Primary Ciliary Dyskinesia and Subjects With Structurally Normal Hearts

DEFF Research Database (Denmark)

Loomba, Rohit S; Danduran, Michael; Nielsen, Kim G

2017-01-01

with and without isomerism. We have now compared these finding with those from patients with primary ciliary dyskinesia, as many patients with isomerism have ciliary dyskinesia. We identified patients having the Fontan circulation with and without isomerism who had undergone cardiopulmonary exercise testing......, comparing the findings from healthy individuals undergoing exercise, and a comparable number of individuals with primary ciliary dyskinesia but no congenital heart disease. We were able to include a total of 68 patients in our study, with 17 in each of the four groups. Cardiopulmonary exercise testing...

Growth, biomass production and photosynthesis of Cenchrus ciliaris L. under Acacia tortilis (Forssk.) Hayne based silvopastoral systems in semi arid tropics.

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Mishra, A K; Tiwari, H S; Bhatt, R K

2010-11-01

The growth, biomass production and photosynthesis of Cenchrus ciliaris was studied under the canopies of 17 yr old Acacia tortilis trees in semi arid tropical environment. On an average the full grown canopy of A. tortilis at the spacing of 4 x 4 m allowed 55% of total Photosynthetically Active Radiation (PAR) which in turn increased Relative Humidity (RH) and reduced under canopy temperature to -1.75 degrees C over the open air temperature. C. ciliaris attained higher height under the shade of A. tortilis. The tiller production and leaf area index decreased marginally under the shade of tree canopies as compared to the open grown grasses. C. ciliaris accumulated higher chlorophyll a and b under the shade of tree canopies indicating its shade adaptation potential. The assimilatory functions such as rate of photosynthesis, transpiration, stomatal conductance, photosynthetic water use efficiency (PN/TR) and carboxylation efficiency (PN/CINT) decreased under the tree canopies due to low availability of PAR. The total biomass production in term of fresh and dry weight decreased under the tree canopies. On average of 2 yr C. ciliaris had produced 12.78 t ha(-1) green and 3.72 -t ha(-1) dry biomass under the tree canopies of A. tortilis. The dry matter yield reduced to 38% under the tree canopies over the open grown grasses. The A. tortilis + C. ciliaris maintained higher soil moisture, organic carbon content and available N P K for sustainable biomass production for the longer period. The higher accumulation of crude protein, starch, sugar and nitrogen in leaves and stem of C. ciliaris indicates that this grass species also maintained its quality under A. tortilis based silvopastoral system. The photosynthesis and dry matter accumulation are closely associated with available PAR indicating that for sustainable production of this grass species in the silvopasture systems for longer period about 55% or more PAR is required.

Primary ciliary dyskinesia: a report from ATS 2001, May 18–23, San Francisco

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Noone Peadar G

2001-06-01

Full Text Available Abstract Primary ciliary dyskinesia (PCD is a genetic disorder of abnormal ciliary structure and function that leads to defective mucociliary clearance, resulting in oto-sino-pulmonary disease, and infertility. The disease is currently under intense investigation by a number of research groups worldwide. At the recent American Thoracic Society meeting in San Francisco in May 2001, two sessions focused on PCD; a symposium session on May 21 with several featured expert speakers was followed by a mini-symposium on Tuesday May 22, with one featured speaker and presentation of nine abstracts covering a range of research topics. Mattias Salathe (University of Miami, USA and Stephen Brody (Washington University, St Louis, USA chaired the symposium session. Presentations focused on the clinical spectrum of PCD, the genetics of PCD, a proteomics approach to detail the structure of cilia, the role of cilia in the embryology of situs laterality, and airway epithelial cell biology. The mini-symposium was chaired by Peadar Noone (University of North Carolina, USA and Malcolm King (University of Alberta, USA and included presentations on the use of PCD as a human disease model, accurate definition of the phenotype using clinical and cell biologic markers, and molecular studies. The latter reports ranged from isolation of a protein involved in ciliary structure and function to genetic studies using linkage analysis and the candidate gene approach. Clinicians and scientists alike displayed considerable interest at both sessions, and there were several lively question–answer sessions.

Ciliary metachronal wave propagation on the compliant surface of Paramecium cells.

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Narematsu, Naoki; Quek, Raymond; Chiam, Keng-Hwee; Iwadate, Yoshiaki

2015-12-01

Ciliary movements in protozoa exhibit metachronal wave-like coordination, in which a constant phase difference is maintained between adjacent cilia. It is at present generally thought that metachronal waves require hydrodynamic coupling between adjacent cilia and the extracellular fluid. To test this hypothesis, we aspirated a Paramecium cell using a micropipette which completely sealed the surface of the cell such that no fluid could pass through the micropipette. Thus, the anterior and the posterior regions of the cell were hydrodynamically decoupled. Nevertheless, we still observed that metachronal waves continued to propagate from the anterior to the posterior ends of the cell, suggesting that in addition to hydrodynamic coupling, there are other mechanisms that can also transmit the metachronal waves. Such transmission was also observed in computational modeling where the fluid was fully decoupled between two partitions of a beating ciliary array. We also imposed cyclic stretching on the surface of live Paramecium cells and found that metachronal waves persisted in the presence of cyclic stretching. This demonstrated that, in addition to hydrodynamic coupling, a compliant substrate can also play a critical role in mediating the propagation of metachronal waves. © 2015 Wiley Periodicals, Inc.

Palladium-103 plaque radiation therapy for ciliary body melanoma through a functioning glaucoma filtering bleb.

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Pathan, Arif H K; Barash, Alexander; Tena, Lawrence B; Finger, Paul T

2018-01-01

To provide a clinical description of the long-term outcome of a 103 Pd plaque-irradiated ciliary body melanoma with extrascleral extension while attempting to preserve a subadjacent glaucoma filtering bleb. A 75-year-old woman with pseudoexfoliative glaucoma for 17 years, 16 years status post argon laser trabeculoplasty, and 15 years status post trabeculectomy in the left eye, was diagnosed with an ipsilateral ciliary body melanoma with visible extrascleral extension. Treatment involved insertion of a 103 Pd radioactive plaque over the functioning trabeculectomy, with removal 7 days later. At plaque insertion, amniotic membrane grafts were used to cover the plaque and protect the filtering site. The tumor was successfully treated without clinical evidence of harm to the filtering bleb, with resultant stable intraocular pressure. However, the patient developed blebitis 1.5 years later. Though it resolved with topical antibiotic therapy, the bleb became less succulent. Two years postoperatively, she developed a spontaneous hyphema that resolved after one injection of transscleral bevacizumab 1.25 mg. Her tumor continually regressed in thickness. Without additional glaucoma surgery, her intraocular pressure remained well-controlled on topical medications for 6 years. Ciliary body melanoma with minimal extrascleral extension beneath a functioning filtering bleb can be treated using radioactive plaque therapy. In this case, we were able to achieve both tumor regression and glaucoma control by covering the plaque with an amniotic membrane graft.

Bug22p, a Conserved Centrosomal/Ciliary Protein Also Present in Higher Plants, Is Required for an Effective Ciliary Stroke in Paramecium ▿ †

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Laligné, C.; Klotz, C.; Garreau de Loubresse, N.; Lemullois, M.; Hori, M.; Laurent, F. X.; Papon, J. F.; Louis, B.; Cohen, J.; Koll, F.

2010-01-01

Centrioles, cilia, and flagella are ancestral conserved organelles of eukaryotic cells. Among the proteins identified in the proteomics of ciliary proteins in Paramecium, we focus here on a protein, Bug22p, previously detected by cilia and basal-body high-throughput studies but never analyzed per se. Remarkably, this protein is also present in plants, which lack centrioles and cilia. Bug22p sequence alignments revealed consensus positions that distinguish species with centrioles/cilia from plants. In Paramecium, antibody and green fluorescent protein (GFP) fusion labeling localized Bug22p in basal bodies and cilia, and electron microscopy immunolabeling refined the localization to the terminal plate of the basal bodies, the transition zone, and spots along the axoneme, preferentially between the membrane and the microtubules. RNA interference (RNAi) depletion of Bug22p provoked a strong decrease in swimming speed, followed by cell death after a few days. High-speed video microscopy and morphological analysis of Bug22p-depleted cells showed that the protein plays an important role in the efficiency of ciliary movement by participating in the stroke shape and rigidity of cilia. The defects in cell swimming and growth provoked by RNAi can be complemented by expression of human Bug22p. This is the first reported case of complementation by a human gene in a ciliate. PMID:20118210

Impact of the Smoothened inhibitor, IPI-926, on smoothened ciliary localization and Hedgehog pathway activity.

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Marisa O Peluso

Full Text Available A requisite step for canonical Hedgehog (Hh pathway activation by Sonic Hedgehog (Shh ligand is accumulation of Smoothened (Smo to the primary cilium (PC. Activation of the Hh pathway has been implicated in a broad range of cancers, and several Smo antagonists are being assessed clinically, one of which is approved for the treatment of advanced basal cell carcinoma. Recent reports demonstrate that various Smo antagonists differentially impact Smo localization to the PC while still exerting inhibitory activity. In contrast to other synthetic small molecule Smo antagonists, the natural product cyclopamine binds to and promotes ciliary accumulation of Smo and "primes" cells for Hh pathway hyper-responsiveness after compound withdrawal. We compared the properties of IPI-926, a semi-synthetic cyclopamine analog, to cyclopamine with regard to potency, ciliary Smo accumulation, and Hh pathway activity after compound withdrawal. Like cyclopamine, IPI-926 promoted accumulation of Smo to the PC. However, in contrast to cyclopamine, IPI-926 treatment did not prime cells for hyper-responsiveness to Shh stimulation after compound withdrawal, but instead demonstrated continuous inhibition of signaling. By comparing the levels of drug-induced ciliary Smo accumulation with the degree of Hh pathway activity after compound withdrawal, we propose that a critical threshold of ciliary Smo is necessary for "priming" activity to occur. This "priming" appears achievable with cyclopamine, but not IPI-926, and is cell-line dependent. Additionally, IPI-926 activity was evaluated in a murine tumor xenograft model and a pharmacokinetic/pharmacodynamic relationship was examined to assess for in vivo evidence of Hh pathway hyper-responsiveness. Plasma concentrations of IPI-926 correlated with the degree and duration of Hh pathway suppression, and pathway activity did not exceed baseline levels out to 96 hours post dose. The overall findings suggest that IPI-926 possesses

Mutations in LCA5, encoding the ciliary protein lebercilin, cause Leber congenital amaurosis.

NARCIS (Netherlands)

Hollander, A.I. den; Koenekoop, R.K.; Mohamed, M.D.; Arts, H.H.; Boldt, K.; Towns, K.V.; Sedmak, T.; Beer, M. de; Nagel-Wolfrum, K.; McKibbin, M.; Dharmaraj, S.; Lopez, I.; Ivings, L.; Williams, G.A.; Springell, K.; Woods, C.G.; Jafri, H.; Rashid, Y.; Strom, T.M.; Zwaag, B. van der; Gosens, I.; Kersten, F.F.J.; Wijk, E. van; Veltman, J.A.; Zonneveld, M.N.; Beersum, S.E.C. van; Maumenee, I.H.; Wolfrum, U.; Cheetham, M.E.; Ueffing, M.; Cremers, F.P.M.; Inglehearn, C.F.; Roepman, R.

2007-01-01

Leber congenital amaurosis (LCA) causes blindness or severe visual impairment at or within a few months of birth. Here we show, using homozygosity mapping, that the LCA5 gene on chromosome 6q14, which encodes the previously unknown ciliary protein lebercilin, is associated with this disease. We

Influence of essential and fatty oils on ciliary beat frequency of human nasal epithelial cells.

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Neher, Andreas; Gstöttner, Michaela; Thaurer, Michael; Augustijns, Patrick; Reinelt, Monika; Schobersberger, Wolfgang

2008-01-01

In alternative and complementary medicine, the use of essential and fatty oils has become more and more popular. In addition to conventional medical therapies, self-medication is showing increasing popularity, using agents with unclear compounds and poorly controlled dosages. Among other disorders, these alternative treatments are used in bronchitis and rhinitis, including some topical applications. Thus, the influence on ciliated epithelia should be evaluated, because a disturbance of the ciliary function can lead to recurrent sinusitis and chronic rhinosinusitis. The aim of this study was to test the influence of fatty and essential oils on the ciliary beat frequency (CBF) of nasal mucosa in vivo. The influence of sesame oil, soy oil, peanut oil, Miglyol 840, thyme oil, lavender oil, eucalyptus oil, and menthol on the ciliary activity of nasal brushings was evaluated by digital high-speed imaging. The presence of most fatty oils resulted in an increase in CBF, the effect being highest for peanut oil. Miglyol 840 had no significant influence on CBF. The essential oils were tested at a concentration of 0.2 and 2%. Thyme oil did not affect CBF, whereas the presence of all other essentials oils resulted in an increase in CBF; the effect was higher at 0.2% than at 2%. Except thyme oil and Miglyol 840, all tested oils caused an increase in CBF. Interestingly, the 0.2% concentrations of essential oils resulted in stronger effects when compared with the 2% concentrations.

Continued administration of ciliary neurotrophic factor protects mice from inflammatory pathology in experimental autoimmune encephalomyelitis

DEFF Research Database (Denmark)

Kuhlmann, Tanja; Remington, Leah; Cognet, Isabelle

2006-01-01

Multiple sclerosis is an inflammatory disease of the central nervous system that leads to loss of myelin and oligodendrocytes and damage to axons. We show that daily administration (days 8 to 24) of murine ciliary neurotrophic factor (CNTF), a neurotrophic factor that has been described as a surv......Multiple sclerosis is an inflammatory disease of the central nervous system that leads to loss of myelin and oligodendrocytes and damage to axons. We show that daily administration (days 8 to 24) of murine ciliary neurotrophic factor (CNTF), a neurotrophic factor that has been described...... it was withdrawn. After cessation of CNTF treatment, inflammation and symptoms returned to control levels. However, slight but significantly higher numbers of oligodendrocytes, NG2-positive cells, axons, and neurons were observed in mice that had been treated with high concentrations of CNTF. Our results show...

Primary ciliary dyskinesia-causing mutations in Amish and Mennonite communities.

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Ferkol, Thomas W; Puffenberger, Erik G; Lie, Hauw; Helms, Cynthia; Strauss, Kevin A; Bowcock, Anne; Carson, John L; Hazucha, Milan; Morton, D Holmes; Patel, Anand C; Leigh, Margaret W; Knowles, Michael R; Zariwala, Maimoona A

2013-08-01

To determine whether individuals with primary ciliary dyskinesia (PCD) from unrelated Amish and Mennonite families harbor a single and unique founder mutation. Subjects from Amish and Mennonite communities in several states were enrolled in the study. All subjects were clinically characterized, and nasal nitric oxide levels were measured. Nasal epithelial scrapings were collected from several subjects for ciliary ultrastructural analyses. DNA was isolated from patients with PCD and their unaffected first- and second-degree relatives. Genome-wide homozygosity mapping, linkage analyses, targeted mutation analyses, and exome sequencing were performed. All subjects from Old-Order Amish communities from Pennsylvania were homozygous for a nonsense mutant DNAH5 allele, c.4348C>T (p.Q1450X). Two affected siblings from an unrelated Mennonite family in Arkansas were homozygous for the same nonsense DNAH5 mutation. Children with PCD from an Amish family from Wisconsin had biallelic DNAH5 mutations, c.4348C>T (p.Q1450X) and c.10815delT (p.P3606HfsX23), and mutations in other genes associated with PCD were also identified in this community. The Amish and Mennonite subjects from geographically dispersed and socially isolated communities had the same founder DNAH5 mutation, owing to the common heritage of these populations. However, disease-causing mutations in other PCD-associated genes were also found in affected individuals in these communities, illustrating the genetic heterogeneity in this consanguineous population. Copyright © 2013 Mosby, Inc. All rights reserved.

A longitudinal evaluation of hearing and ventilation tube insertion in patients with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Andersen, Tobias Nicolai; Alanin, Mikkel Christian; von Buchwald, Christian

2016-01-01

INTRODUCTION: Primary ciliary dyskinesia (PCD) is an autosomal recessive genetic disease, which primarily manifests with oto-sino-pulmonary symptoms. Otitis media with effusion (OME) is common from early childhood. The existing literature on OME management in PCD is conflicting. The goals of the ...

Ahmed glaucoma valve implantation with tube insertion through the ciliary sulcus in pseudophakic/aphakic eyes.

Science.gov (United States)

Eslami, Yadolla; Mohammadi, Massood; Fakhraie, Ghasem; Zarei, Reza; Moghimi, Sasan

2014-02-01

To report the efficacy and safety of Ahmed glaucoma valve (AGV) insertion into the ciliary sulcus in pseudophakic/aphakic patients. A chart review was done on patients with uncontrolled glaucoma, who underwent AGV implantation with tube inserted into the ciliary sulcus. Baseline intraocular pressure (IOP) and number of medications were compared with that of postoperative follow-up visits. Surgical success was defined as last IOP glaucoma control, and without loss of light perception. Postoperative complications were recorded. Twenty-three eyes of 23 patients were recruited with the mean follow-up of 9 months (range, 3 to 24 mo). The mean (SD) age of patients was 49.9 (16.9) years (range, 22 to 80 years). The mean (SD) IOP (mm Hg) was reduced from 37.9 (12.4) before surgery to 16.2 (3.6) at the last follow-up visit (P<0.001). The mean (SD) number of medications was reduced from 3.3 (0.9) preoperatively to 1 (1.1) at the last follow-up (P<0.001). Success rate was 18/23 (78.6%). Complications included endophthalmitis in 1 eye, tube exposure in 1 diabetic patient, and vitreous tube occlusion in 1 eye. No case of corneal decompensation or graft failure was seen during follow-up. Ciliary sulcus placement of the tube of AGV effectively reduces IOP and medication use in short term. It has the potential to lower corneal complications of anterior chamber tube insertion and avoids the need for pars plana vitrectomy and tube insertion in patients at higher risk of corneal decompensation.

Long-term outcomes of ciliary sulcus versus capsular bag fixation of intraocular lenses in children: An ultrasound biomicroscopy study.

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Yun-E Zhao

Full Text Available To evaluate the long-term outcomes of ciliary sulcus versus capsular bag fixation of intraocular lenses (IOLs in children after pediatric cataract surgery.IOL was implanted in the ciliary sulcus in 21 eyes of 14 children, and in the capsular bag in 19 eyes of 12 children for the treatment of pediatric cataract in an institutional setting. Ultrasound biomicroscopy (UBM was performed. Main outcome measures included IOL decentration, IOL tilt, anterior chamber depth (ACD, angle-opening distance at 500 μm (AOD500, trabecular-iris angle (TIA, best-corrected visual acuity (BCVA, intraocular pressure (IOP, and incidence of postoperative complications.The mean follow-up period was 6.81 ± 1.82 years. Comparing to the capsular bag fixation group, the ciliary sulcus fixation group had higher vertical IOL decentration, horizontal IOL tilt, and vertical IOL tilt (p = 0.02, 0.01,0.01, respectively, higher incidence of iris-IOL contact and peripheral anterior synechia (p = 0.001, 0.03, respectively, smaller ACD, AOD500, and TIA (p = 0.02, 0.03, 0.04, respectively, higher mean IOP (17.10 ±6.06 mmHg vs.14.15± 4.74 mmHg, p = 0.01, and higher incidence of secondary glaucoma (28.57% vs. 10.53%, p = 0.007.There was no significant difference between the two groups with regard to the BCVA, refractive errors, incidence of myopic shift, nystagmus, strabismus, and visual axis opacity.Ciliary sulcus fixation of IOLs in pediatric eyes may increase IOL malposition and crowding of the anterior segment, and may associate with a higher risk of secondary glaucoma compared to capsular bag fixation of IOLs.

Effects of perfluorooctane sulfonate on tracheal ciliary beating frequency in mice

International Nuclear Information System (INIS)

Matsubara, Eriko; Nakahari, Takashi; Yoshida, Hideyo; Kuroiwa, Toshihiko; Harada, Kouji H.; Inoue, Kayoko; Koizumi, Akio

2007-01-01

Perfluorooctane sulfonate (PFOS) is one of the emerging persistent organic pollutants, ubiquitously found in the global environment, even in human serum. PFOS has been reported to perturb Ca 2+ homeostasis in Paramecium, cardiomyocytes and neurons. Since ciliary beat frequency (CBF) in the trachea is known to be increased by cytoplasmic Ca 2+ elevation, the effects of PFOS on CBF were evaluated in a slice preparation using video-enhanced contrast microscopy. PFOS increased CBF by 11% (P 2+ concentration ([Ca 2+ ] i ) in mouse tracheal ciliary cells. In Ca 2+ -free solution, PFOS at 100 μM failed to increase CBF (0.96-fold of vehicle control). The addition of Gd 3+ (1 μM), a store-operated Ca 2+ channel blocker, did not prevent the increase in CBF (1.09-fold (P + concentration (50 mM), which causes depolarization of the plasma membrane potential and a transient increase in [Ca 2+ ] i , increased CBF by 20% (P 2+ channels (VDCCs) in stimulation of CBF. Nifedipine (30 μM), a selective VDCC blocker, antagonized the effects of high K + (0.92-fold of high K + solution) and PFOS (0.96-fold of vehicle control) on CBF. In cells from peroxisome proliferator-activated receptor α (PPARα)-null mice, PFOS still increased CBF (1.12-fold (P 2+ through VDCC

An international registry for primary ciliary dyskinesia

DEFF Research Database (Denmark)

Werner, Claudius; Lablans, Martin; Ataian, Maximilian

2016-01-01

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder leading to chronic upper and lower airway disease. Fundamental data on epidemiology, clinical presentation, course and treatment strategies are lacking in PCD. We have established an international PCD registry to realise...... an unmet need for an international platform to systematically collect data on incidence, clinical presentation, treatment and disease course.The registry was launched in January 2014. We used internet technology to ensure easy online access using a web browser under www.pcdregistry.eu. Data from 201...... methods in addition to classical clinical symptoms. Preliminary analysis of lung function data demonstrated a mean annual decline of percentage predicted forced expiratory volume in 1 s of 0.59% (95% CI 0.98-0.22).Here, we present the development of an international PCD registry as a new promising tool...

Localization of a guanylyl cyclase to chemosensory cilia requires the novel ciliary MYND domain protein DAF-25.

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Victor L Jensen

2010-11-01

Full Text Available In harsh conditions, Caenorhabditis elegans arrests development to enter a non-aging, resistant diapause state called the dauer larva. Olfactory sensation modulates the TGF-β and insulin signaling pathways to control this developmental decision. Four mutant alleles of daf-25 (abnormal DAuer Formation were isolated from screens for mutants exhibiting constitutive dauer formation and found to be defective in olfaction. The daf-25 dauer phenotype is suppressed by daf-10/IFT122 mutations (which disrupt ciliogenesis, but not by daf-6/PTCHD3 mutations (which prevent environmental exposure of sensory cilia, implying that DAF-25 functions in the cilia themselves. daf-25 encodes the C. elegans ortholog of mammalian Ankmy2, a MYND domain protein of unknown function. Disruption of DAF-25, which localizes to sensory cilia, produces no apparent cilia structure anomalies, as determined by light and electron microscopy. Hinting at its potential function, the dauer phenotype, epistatic order, and expression profile of daf-25 are similar to daf-11, which encodes a cilium-localized guanylyl cyclase. Indeed, we demonstrate that DAF-25 is required for proper DAF-11 ciliary localization. Furthermore, the functional interaction is evolutionarily conserved, as mouse Ankmy2 interacts with guanylyl cyclase GC1 from ciliary photoreceptors. The interaction may be specific because daf-25 mutants have normally-localized OSM-9/TRPV4, TAX-4/CNGA1, CHE-2/IFT80, CHE-11/IFT140, CHE-13/IFT57, BBS-8, OSM-5/IFT88, and XBX-1/D2LIC in the cilia. Intraflagellar transport (IFT (required to build cilia is not defective in daf-25 mutants, although the ciliary localization of DAF-25 itself is influenced in che-11 mutants, which are defective in retrograde IFT. In summary, we have discovered a novel ciliary protein that plays an important role in cGMP signaling by localizing a guanylyl cyclase to the sensory organelle.

Multicenter analysis of body mass index, lung function, and sputum microbiology in primary ciliary dyskinesia

DEFF Research Database (Denmark)

Maglione, Marco; Bush, Andrew; Nielsen, Kim G

2014-01-01

BACKGROUND: No studies longitudinally, simultaneously assessed body mass index (BMI) and spirometry in primary ciliary dyskinesia (PCD). METHODS: We determined BMI and spirometry in 158 PCD children and adolescents from London, UK (n = 75), Naples, Italy (n = 23) and Copenhagen, Denmark (n = 60) ...

hemingway is required for sperm flagella assembly and ciliary motility in Drosophila.

Science.gov (United States)

Soulavie, Fabien; Piepenbrock, David; Thomas, Joëlle; Vieillard, Jennifer; Duteyrat, Jean-Luc; Cortier, Elisabeth; Laurençon, Anne; Göpfert, Martin C; Durand, Bénédicte

2014-04-01

Cilia play major functions in physiology and development, and ciliary dysfunctions are responsible for several diseases in humans called ciliopathies. Cilia motility is required for cell and fluid propulsion in organisms. In humans, cilia motility deficiencies lead to primary ciliary dyskinesia, with upper-airways recurrent infections, left-right asymmetry perturbations, and fertility defects. In Drosophila, we identified hemingway (hmw) as a novel component required for motile cilia function. hmw encodes a 604-amino acid protein characterized by a highly conserved coiled-coil domain also found in the human orthologue, KIAA1430. We show that HMW is conserved in species with motile cilia and that, in Drosophila, hmw is expressed in ciliated sensory neurons and spermatozoa. We created hmw-knockout flies and found that they are hearing impaired and male sterile. hmw is implicated in the motility of ciliated auditory sensory neurons and, in the testis, is required for elongation and maintenance of sperm flagella. Because HMW is absent from mature flagella, we propose that HMW is not a structural component of the motile axoneme but is required for proper acquisition of motile properties. This identifies HMW as a novel, evolutionarily conserved component necessary for motile cilium function and flagella assembly.

C2 Domains as Protein-Protein Interaction Modules in the Ciliary Transition Zone

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Kim Remans

2014-07-01

Full Text Available RPGR-interacting protein 1 (RPGRIP1 is mutated in the eye disease Leber congenital amaurosis (LCA and its structural homolog, RPGRIP1-like (RPGRIP1L, is mutated in many different ciliopathies. Both are multidomain proteins that are predicted to interact with retinitis pigmentosa G-protein regulator (RPGR. RPGR is mutated in X-linked retinitis pigmentosa and is located in photoreceptors and primary cilia. We solved the crystal structure of the complex between the RPGR-interacting domain (RID of RPGRIP1 and RPGR and demonstrate that RPGRIP1L binds to RPGR similarly. RPGRIP1 binding to RPGR affects the interaction with PDEδ, the cargo shuttling factor for prenylated ciliary proteins. RPGRIP1-RID is a C2 domain with a canonical β sandwich structure that does not bind Ca2+ and/or phospholipids and thus constitutes a unique type of protein-protein interaction module. Judging from the large number of C2 domains in most of the ciliary transition zone proteins identified thus far, the structure presented here seems to constitute a cilia-specific module that is present in multiprotein transition zone complexes.

Genotypic variation for salinity tolerance in Cenchrus ciliaris L

Directory of Open Access Journals (Sweden)

M. Iftikhar Hussain

2016-07-01

Full Text Available Scarcity of irrigation water and increasing soil salinization has threatened the sustainability of forage production in arid and semi-arid region around the globe. Introduction of salt-tolerant perennial species is a promising alternative to overcome forage deficit to meet future livestock needs in salt-affected areas. This study presents the results of a salinity tolerance screening trial which was carried out in plastic pots buried in the open field for 160 buffelgrass (Cenchrus ciliaris L. accessions for three consecutive years (2003-2005. The plastic pots were filled with sand, organic, and peat moss mix and were irrigated with four different quality water (EC 0, 10, 15, and 20 dS m-1. The results indicate that the average annual dry weights (DW were in the range from 122.5 – 148.9 g pot-1 in control; 96.4 – 133.8 g pot-1 at 10 dS m-1; 65.6 – 80.4 g pot-1 at 15 dS m-1, and 55.4- 65.6 g pot-1 at 20 dS m-1. The highest DW (148.9 g pot-1 was found with accession 49 and the lowest with accession 23. Principle component analysis shows that PC-1 contributed 81.8 % of the total variability, while PC-2 depicted 11.7% of the total variation among C. ciliaris accessions for DW. Hierarchical cluster analysis revealed that a number of accessions collected from diverse regions could be grouped into a single cluster. Accessions 3, 133, 159, 30, 23, 142, 141, 95, 49, 129, 124, and 127 were stable, salt tolerant, and produced good dry biomass yield. These accessions demonstrate sufficient salinity tolerance potential for promotion in marginal land and arid regions to enhance farm productivity and reduce rural poverty.

Synergism between sulfur dioxide and carbon particles. Studies on adsorption and on ciliary movements in the rabbit trachea in vivo

Energy Technology Data Exchange (ETDEWEB)

Dalhamn, T; Strandberg, L

1963-01-01

Various types of carbon were shown to absorb gaseous SO/sub 2/ (about 75% at equilibrium in one case), which was rapidly converted to sulfuric acid (30% of that adsorbed in one case). However, carbon did not act synergistically with SO/sub 2/ in reducing rabbit trachea ciliary beat; carbon had no effect by itself and had no additional effect when administered with SO/sub 2/. 74 to 134 ppM SO/sub 2/ reduced ciliary beat from 1265 to 1091 beats/min after 45 min, and 175 to 239 ppM SO/sub 2/ reduced it from 1200 to 891 beats/min after 45 min.

Short Communication: An apospory-specific genomic region is conserved between Buffelgrass (Cenchrus ciliaris L.) and Pennisetum squamulatum Fresen.

Science.gov (United States)

Roche; Cong; Chen; Hanna; Gustine; Sherwood; Ozias-Akins

1999-07-01

Twelve molecular markers linked to pseudogamous apospory, a form of gametophytic apomixis, were previously isolated from Pennisetum squamulatum Fresen. No recombination between these markers was found in a segregating population of 397 individuals (Ozias-Akins et al. 1998, Proc. Natl Acad. Sci. USA, 95, 5127-5132). The objective of the present study was to test if these markers were also linked to the aposporous mode of reproduction in two small segregating populations of Cenchrus ciliaris (= Pennisetum ciliare (L.)Link), another apomictic grass species. Among 12 markers (sequence characterized amplified regions, SCARs), six were scored as dominant markers between aposporous and sexual C. ciliaris genotypes (presence/absence, respectively). Five were always linked to apospory and one showed a low level of recombination in 84 progenies. Restriction fragment length polymorphisms (RFLPs) were observed between sexual and apomictic phenotypes for three of the six remaining SCARs from P. squamulatum when used as probes. No recombination was observed in the F1 progenies. Preliminary data from megabase DNA analysis and sequencing in both species indicate that an apospory-specific genomic region (ASGR) is highly conserved between the two species. Although C. ciliaris has a smaller genome size to P. squamulatum, a higher copy number for markers linked to apospory found in the former may impair the progress of positional cloning of gene(s) for apomixis in this species.

Clinical value of measurement of pulmonary radioaerosol mucociliary clearance in the work up of primary ciliary dyskinesia

DEFF Research Database (Denmark)

Munkholm, Mathias; Nielsen, Kim Gjerum; Mortensen, Jann

2015-01-01

BACKGROUND: We aimed to evaluate and define the general clinical applicability and impact of pulmonary radioaerosol mucociliary clearance (PRMC) on the work up of patients suspected of having primary ciliary dyskinesia (PCD). In addition, we wanted to evaluate the accuracy of the reference values...... primarily to results from nasal ciliary function testing, to electron microscopic (EM) examination of the ultrastructure of the cilia, and to the final clinical diagnosis. RESULTS: Of the 239 patients, 27 ended up with a final clinical diagnosis of definitive PCD. No patients with a PRMC test...... of the entire lung. Its greatest strength is its ability to reject a suspected PCD diagnosis with great certainty. In our material, this accounted for 2/3 of referred patients. In addition, the test has a high rate of conclusive results. According to our analyses, reference equations on children would benefit...

CCDC151 Mutations Cause Primary Ciliary Dyskinesia by Disruption of the Outer Dynein Arm Docking Complex Formation

NARCIS (Netherlands)

Hjeij, R.; Onoufriadis, A.; Watson, C.M.; Slagle, C.E.; Klena, N.T.; Dougherty, G.W.; Kurkowiak, M.; Loges, N.T.; Diggle, C.P.; Morante, N.F.; Gabriel, G.C.; Lemke, K.L.; Li, Y.; Pennekamp, P.; Menchen, T.; Konert, F.; Marthin, J.K.; Mans, D.A.; Letteboer, S.J.F.; Werner, C.; Burgoyne, T.; Westermann, C.; Rutman, A.; Carr, I.M.; O'Callaghan, C.; Moya, E.; Chung, E.M.; Consortium, U.K.; Sheridan, E.; Nielsen, K.G.; Roepman, R.; Bartscherer, K.; Burdine, R.D.; Lo, C.W.; Omran, H.; Mitchison, H.M.

2014-01-01

A diverse family of cytoskeletal dynein motors powers various cellular transport systems, including axonemal dyneins generating the force for ciliary and flagellar beating essential to movement of extracellular fluids and of cells through fluid. Multisubunit outer dynein arm (ODA) motor complexes,

Ultrastructural alterations in ciliary cells exposed to ionizing radiation. A scanning and transmission electron microscopic study

Energy Technology Data Exchange (ETDEWEB)

Baldetorp, L; Mecklenburg, C v; Haakansson, C H [Lund Univ. (Sweden). Hospital; Lund Univ. (Sweden). Dept. of Zoology)

1977-01-01

Early effects of ionizing radiation were investigated in an experimental in vitro system using the ciliary cells of the tracheal mucous membrane of the rabbit, irradiated at 30/sup 0/C and at more than 90% humidity. The changes in physiological activities of the ciliary cells caused by irradation were continously registered during the irradation. The specimens were examined immediately after irradiation electron microscopically. The morphological changes in irradiated material after 10-70 Gy are compared with normal material. After 40-70 Gy, scanning electron microscopy revealed the formation of vesicles on cilia, and club-like protrusions and adhesion of their tips. After 30-70 Gy, a swelling of mitochondrial membranes and cristae was apparent transmission electron microscopically. The membrane alterations caused by irradiation are assumed to disturb the permeability and flow of ATP from the mitochondria, which in turn leads to the recorded changes in the activity of the ciliated cells.

Measurement of ciliary beat frequency using Doppler optical coherence tomography.

Science.gov (United States)

Lemieux, Bryan T; Chen, Jason J; Jing, Joseph; Chen, Zhongping; Wong, Brian J F

2015-11-01

Measuring ciliary beat frequency (CBF) is a technical challenge and difficult to perform in vivo. Doppler optical coherence tomography (D-OCT) is a mesoscopic noncontact imaging modality that provides high-resolution tomographic images and detects micromotion simultaneously in living tissues. In this work we used D-OCT to measure CBF in ex vivo tissue as the first step toward translating this technology to clinical use. Fresh ex vivo samples of rabbit tracheal mucosa were imaged using both D-OCT and phase-contrast microscopy (n = 5). The D-OCT system was designed and built to specification in our lab (1310-nm swept source vertical-cavity surface-emitting laser [VCSEL], 6-μm axial resolution). The samples were placed in culture and incubated at 37°C. A fast Fourier transform was performed on the D-OCT signal recorded on the surface of the samples to gauge CBF. High-speed digital video of the epithelium recorded via phase-contrast microscopy was analyzed to confirm the CBF measurements. The D-OCT system detected Doppler signal at the epithelial layer of ex vivo rabbit tracheal samples suggestive of ciliary motion. CBF was measured at 9.36 ± 1.22 Hz using D-OCT and 9.08 ± 0.48 Hz using phase-contrast microscopy. No significant differences were found between the 2 methods (p > 0.05). D-OCT allows for the quantitative measurement of CBF without the need to resolve individual cilia. Furthermore, D-OCT technology can be incorporated into endoscopic platforms that allow clinicians to readily measure CBF in the office and provide a direct measurement of mucosal health. © 2015 ARS-AAOA, LLC.

CCDC151 mutations cause primary ciliary dyskinesia by disruption of the outer dynein arm docking complex formation

DEFF Research Database (Denmark)

Hjeij, Rim; Onoufriadis, Alexandros; Watson, Christopher M

2014-01-01

disorder of ciliary and flagellar dysmotility characterized by chronic upper and lower respiratory infections and defects in laterality. Here, by combined high-throughput mapping and sequencing, we identified CCDC151 loss-of-function mutations in five affected individuals from three independent families...

The role of hair cells, cilia and ciliary motility in otolith formation in the zebrafish otic vesicle.

Science.gov (United States)

Stooke-Vaughan, Georgina A; Huang, Peng; Hammond, Katherine L; Schier, Alexander F; Whitfield, Tanya T

2012-05-01

Otoliths are biomineralised structures required for the sensation of gravity, linear acceleration and sound in the zebrafish ear. Otolith precursor particles, initially distributed throughout the otic vesicle lumen, become tethered to the tips of hair cell kinocilia (tether cilia) at the otic vesicle poles, forming two otoliths. We have used high-speed video microscopy to investigate the role of cilia and ciliary motility in otolith formation. In wild-type ears, groups of motile cilia are present at the otic vesicle poles, surrounding the immotile tether cilia. A few motile cilia are also found on the medial wall, but most cilia (92-98%) in the otic vesicle are immotile. In mutants with defective cilia (iguana) or ciliary motility (lrrc50), otoliths are frequently ectopic, untethered or fused. Nevertheless, neither cilia nor ciliary motility are absolutely required for otolith tethering: a mutant that lacks cilia completely (MZovl) is still capable of tethering otoliths at the otic vesicle poles. In embryos with attenuated Notch signalling [mindbomb mutant or Su(H) morphant], supernumerary hair cells develop and otolith precursor particles bind to the tips of all kinocilia, or bind directly to the hair cells' apical surface if cilia are absent [MZovl injected with a Su(H)1+2 morpholino]. However, if the first hair cells are missing (atoh1b morphant), otolith formation is severely disrupted and delayed. Our data support a model in which hair cells produce an otolith precursor-binding factor, normally localised to tether cell kinocilia. We also show that embryonic movement plays a minor role in the formation of normal otoliths.

(/sup 3/H)ouabain localization of Na-K ATPase in the epithelium of rabbit ciliary body pars plicata

Energy Technology Data Exchange (ETDEWEB)

Usukura, J.; Fain, G.L.; Bok, D.

1988-04-01

The secretion of the aqueous humor has been proposed to occur as the result of active Na+ transport by a ouabain-sensitive Na-K ATPase. We have examined the localization of this enzyme in the epithelium of rabbit ciliary body pars plicata using (3H)ouabain autoradiography. Single ciliary processes were isolated and incubated in Ringer containing (3H)ouabain. Processes were then rapidly frozen, freezedried, sectioned and exposed for autoradiography. In the light microscope, silver grains were found predominantly over the nonpigmented epithelial cells. In the electron microscope, grains could be localized for the most part to the interdigitations of the nonpigmented cell basolateral membrane. Label could also be observed at a much lower density above other membranes and above the pigmented and nonpigmented cell cytoplasm. No label was found in sections of control tissue which had been incubated in (3H)ouabain with an excess of cold ouabain. To show that the (3H)ouabain had free access to all of the membrane surfaces within the epithelium, in parallel experiments we incubated isolated processes in horseradish peroxidase. Our experiments suggest that most of the active Na+ transport in ciliary body epithelium occurs across the basolateral membrane of nonpigmented cells into the posterior chamber. Furthermore, the placement of the Na-K ATPase within the narrow membrane infoldings of the interdigitations is consistent with a role for this enzyme in water transport and the production of the aqueous.

A case report of primary ciliary dyskinesia, laterality defects and developmental delay caused by the co-existence of a single gene and chromosome disorder.

LENUS (Irish Health Repository)

Casey, Jillian P

2015-01-01

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder characterised by abnormal ciliary motion and impaired mucociliary clearance, leading to recurrent respiratory infections, sinusitis, otitis media and male infertility. Some patients also have laterality defects. We recently reported the identification of three disease-causing PCD genes in the Irish Traveller population; RSPH4A, DYX1C1 and CCNO. We have since assessed an additional Irish Traveller family with a complex phenotype involving PCD who did not have any of the previously identified PCD mutations.

Central Retinal and Posterior Ciliary Artery Occlusion After Intralesional Injection of Sclerosant to Glabellar Subcutaneous Hemangioma

International Nuclear Information System (INIS)

Matsuo, Toshihiko; Fujiwara, Hiroyasu; Gobara, Hideo; Mimura, Hidefumi; Kanazawa, Susumu

2009-01-01

The aim of this study is to describe vision loss caused by central retinal artery and posterior ciliary artery occlusion as a consequence of sclerotherapy with a polidocanol injection to a glabellar hemangioma. An 18-year-old man underwent direct injection with a 23-gauge needle of 1 mL of a polidocanol-carbon dioxide emulsion into the glabellar subcutaneous hemangioma under ultrasound visualization of the needle tip by radiologists. He developed lid swelling the next day, and 3 days later at referral, the visual acuity in the left eye was no light perception. Funduscopy revealed central retinal artery occlusion and fluorescein angiography disclosed no perfusion at all in the left fundus, indicating concurrent posterior ciliary artery occlusion. The patient also showed mydriasis, blepharoptosis, and total external ophthalmoplegia on the left side. Magnetic resonance imaging demonstrated the swollen medial rectus muscle. In a month, blepharoptosis and ophthalmoplegia resolved but the visual acuity remained no light perception. Sclerosing therapy for facial hemangioma may develop a severe complication such as permanent visual loss.

Posterior Ciliary Artery Occlusion Caused by Hyaluronic Acid Injections Into the Forehead: A Case Report.

Science.gov (United States)

Hu, Xiu Zhuo; Hu, Jun Yan; Wu, Peng Sen; Yu, Sheng Bo; Kikkawa, Don O; Lu, Wei

2016-03-01

Although cosmetic facial soft tissue fillers are generally safe and effective, improper injections can lead to devastating and irreversible consequences. We represent the first known case of posterior ciliary artery occlusion caused by hyaluronic acid. A 41-year-old female presented with right visual loss 7 hours after receiving cosmetic hyaluronic acid injections into her forehead. Examination revealed no light perception in the right eye and multiple dark ischemic area of injection over the forehead and nose. The right fundus revealed a pink retina with optic nerve edema. Fluorescein angiogram showed several filling defects in the choroidal circulation and late hyperfluorescence in the choroid. A right posterior ciliary artery occlusion and embolic occlusion of facial artery braches was diagnosed. With hyaluronidase injection, hyperbaric oxygen therapy, oral aspirin, oral acetazolamide and dexamethasone venotransfuse treatment, the patient's forehead and nasal skin improved and vision recovered to hand movements. With proper technique, vascular occlusion is rare following facial filler injection. Vision consequences can be severe if filler emboli enter the ocular circulation. Physicians should be aware of this potential side effect, recognize its presentation, and be knowledgeable of effective management.

Genotypic variation in response to salinity in a new sexual germplasm of Cenchrus ciliaris L.

Science.gov (United States)

Quiroga, Mariana; Tommasino, Exequiel; Griffa, Sabrina; Ribotta, Andrea; Colomba, Eliana López; Carloni, Edgardo; Grunberg, Karina

2016-05-01

As part of a breeding program for new salt-tolerant sexual genotypes of Cenchrus ciliaris L., here we evaluated the salt-stress response of two new sexual hybrids, obtained by controlled crosses, at seedling and germination stages. A seedling hydroponic experiment with 300 mM NaCl was performed and physiological variables and growth components were evaluated. While salt-treated sexual material did not show a decrease in productivity with respect to control plants, a differential response in some physiological characteristics was observed. Sexual hybrid 1-9-1 did not suffer oxidative damage and its proline content did not differ from that of control treatment. By contrast, sexual hybrid 1-7-11 suffered oxidative damage and accumulated proline, maintaining its growth under saline stress. At the germination stage, sexual hybrid 1-9-1 presented the highest Germination Rate Index at the maximum NaCl concentration assayed, suggesting an ecological advantage in this genotype. These new sexual resources are promising maternal parental with differential response to salt and could be incorporated in a breeding program of C. ciliaris in the search of new genotypes tolerant to salinity. Copyright © 2016 Elsevier Masson SAS. All rights reserved.

Sonic Hedgehog dependent phosphorylation by CK1α and GRK2 is required for ciliary accumulation and activation of smoothened.

Directory of Open Access Journals (Sweden)

Yongbin Chen

2011-06-01

Full Text Available Hedgehog (Hh signaling regulates embryonic development and adult tissue homeostasis through the GPCR-like protein Smoothened (Smo, but how vertebrate Smo is activated remains poorly understood. In Drosophila, Hh dependent phosphorylation activates Smo. Whether this is also the case in vertebrates is unclear, owing to the marked sequence divergence between vertebrate and Drosophila Smo (dSmo and the involvement of primary cilia in vertebrate Hh signaling. Here we demonstrate that mammalian Smo (mSmo is activated through multi-site phosphorylation of its carboxyl-terminal tail by CK1α and GRK2. Phosphorylation of mSmo induces its active conformation and simultaneously promotes its ciliary accumulation. We demonstrate that graded Hh signals induce increasing levels of mSmo phosphorylation that fine-tune its ciliary localization, conformation, and activity. We show that mSmo phosphorylation is induced by its agonists and oncogenic mutations but is blocked by its antagonist cyclopamine, and efficient mSmo phosphorylation depends on the kinesin-II ciliary motor. Furthermore, we provide evidence that Hh signaling recruits CK1α to initiate mSmo phosphorylation, and phosphorylation further increases the binding of CK1α and GRK2 to mSmo, forming a positive feedback loop that amplifies and/or sustains mSmo phosphorylation. Hence, despite divergence in their primary sequences and their subcellular trafficking, mSmo and dSmo employ analogous mechanisms for their activation.

STOCHASTIC SIMULATION FOR BUFFELGRASS (Cenchrus ciliaris L.) PASTURES IN MARIN, N. L., MEXICO

OpenAIRE

José Romualdo Martínez-López; Erasmo Gutierrez-Ornelas; Miguel Angel Barrera-Silva; Rafael Retes-López

2014-01-01

A stochastic simulation model was constructed to determine the response of net primary production of buffelgrass (Cenchrus ciliaris L.) and its dry matter intake by cattle, in Marín, NL, México. Buffelgrass is very important for extensive livestock industry in arid and semiarid areas of northeastern Mexico. To evaluate the behavior of the model by comparing the model results with those reported in the literature was the objective in this experiment. Model simulates the monthly production of...

Flagellar Synchronization Is a Simple Alternative to Cell Cycle Synchronization for Ciliary and Flagellar Studies.

Science.gov (United States)

Dutta, Soumita; Avasthi, Prachee

2017-01-01

The unicellular green alga Chlamydomonas reinhardtii is an ideal model organism for studies of ciliary function and assembly. In assays for biological and biochemical effects of various factors on flagellar structure and function, synchronous culture is advantageous for minimizing variability. Here, we have characterized a method in which 100% synchronization is achieved with respect to flagellar length but not with respect to the cell cycle. The method requires inducing flagellar regeneration by amputation of the entire cell population and limiting regeneration time. This results in a maximally homogeneous distribution of flagellar lengths at 3 h postamputation. We found that time-limiting new protein synthesis during flagellar synchronization limits variability in the unassembled pool of limiting flagellar protein and variability in flagellar length without affecting the range of cell volumes. We also found that long- and short-flagella mutants that regenerate normally require longer and shorter synchronization times, respectively. By minimizing flagellar length variability using a simple method requiring only hours and no changes in media, flagellar synchronization facilitates the detection of small changes in flagellar length resulting from both chemical and genetic perturbations in Chlamydomonas . This method increases our ability to probe the basic biology of ciliary size regulation and related disease etiologies. IMPORTANCE Cilia and flagella are highly conserved antenna-like organelles that found in nearly all mammalian cell types. They perform sensory and motile functions contributing to numerous physiological and developmental processes. Defects in their assembly and function are implicated in a wide range of human diseases ranging from retinal degeneration to cancer. Chlamydomonas reinhardtii is an algal model system for studying mammalian cilium formation and function. Here, we report a simple synchronization method that allows detection of small

X-linked primary ciliary dyskinesia due to mutations in the cytoplasmic axonemal dynein assembly factor PIH1D3.

NARCIS (Netherlands)

Olcese, C.; Patel, M.P.; Shoemark, A.; Kiviluoto, S.; Legendre, M.; Williams, H.J.; Vaughan, C.K.; Hayward, J.; Goldenberg, A.; Emes, R.D.; Munye, M.M.; Dyer, L.; Cahill, T.; Bevillard, J.; Gehrig, C.; Guipponi, M.; Chantot, S.; Duquesnoy, P.; Thomas, L.; Jeanson, L.; Copin, B.; Tamalet, A.; Thauvin-Robinet, C.; Papon, J.F.; Garin, A.; Pin, I.; Vera, G.; Aurora, P.; Fassad, M.R.; Jenkins, L.; Boustred, C.; Cullup, T.; Dixon, M.; Onoufriadis, A.; Bush, A.; Chung, E.M.; Antonarakis, S.E.; Loebinger, M.R.; Wilson, R.; Armengot, M.; Escudier, E.; Hogg, C.; Amselem, S.; Sun, Z.; Bartoloni, L.; Blouin, J.L.; Mitchison, H.M.; Schmidts, M.; et al.,

2017-01-01

By moving essential body fluids and molecules, motile cilia and flagella govern respiratory mucociliary clearance, laterality determination and the transport of gametes and cerebrospinal fluid. Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder frequently caused by non-assembly of

SURGICAL INTERVENTION AND ACCOMMODATIVE RESPONSES: I. CENTRIPETAL CILIARY BODY, CAPSULE AND LENS MOVEMENT IN RHESUS MONKEYS OF VARYING AGE

Science.gov (United States)

Croft, Mary Ann; Mcdonald, Jared P.; James, Rebecca J.; Heatley, Gregg A.; Lin, Ting-Li; Lütjen-Drecoll, Elke; Kaufman, Paul L.

2009-01-01

Purpose To determine how surgically altering the normal relationship between the lens and the ciliary body in rhesus monkeys affects centripetal ciliary body and lens movement. Methods In 18 rhesus monkey eyes (aged 6–27 years), accommodation was induced before and after surgery by electrical stimulation of the Edinger-Westphal (E–W) nucleus. Accommodative amplitude was measured by coincidence refractometry. Goniovideography was performed before and after intra- and extra-capsular lens extraction (ICLE, ECLE) and anterior regional zonulolysis. Centripetal lens/capsule movements, centripetal ciliary process (CP) movements, and circumlental space were measured by computerized image analysis of the goniovideography images. Results Centripetal accommodative CP and capsule movement increased in velocity and amplitude post-ECLE compared to pre-ECLE regardless of age (n=5). The presence of the lens substance retarded capsule movement by ~21% in the young eyes and by ~62% in the older eyes. Post-ICLE compared to pre-ICLE centripetal accommodative CP movement was dampened in all eyes in which the anterior vitreous was disturbed (n=7), but not in eyes in which the anterior vitreous was left intact (n=2). Following anterior regional zonulolysis (n=4), lens position shifted toward the lysed quadrant during accommodation. Conclusions The presence of the lens substance, capsule zonular attachments, and Wiegers ligament may play a role in centripetal CP movement. The capsule is still capable of centripetal movement in the older eye (although at a reduced capacity) and may have the ability to produce ~6 diopters of accommodation in the presence of a normal young crystalline lens or a similar surrogate. PMID:18552393

Disruption of a Ciliary B9 Protein Complex Causes Meckel Syndrome

Science.gov (United States)

Dowdle, William E.; Robinson, Jon F.; Kneist, Andreas; Sirerol-Piquer, M. Salomé; Frints, Suzanna G.M.; Corbit, Kevin C.; Zaghloul, Norran A.; van Lijnschoten, Gesina; Mulders, Leon; Verver, Dideke E.; Zerres, Klaus; Reed, Randall R.; Attié-Bitach, Tania; Johnson, Colin A.; García-Verdugo, José Manuel; Katsanis, Nicholas; Bergmann, Carsten; Reiter, Jeremy F.

2011-01-01

Nearly every ciliated organism possesses three B9 domain-containing proteins: MKS1, B9D1, and B9D2. Mutations in human MKS1 cause Meckel syndrome (MKS), a severe ciliopathy characterized by occipital encephalocele, liver ductal plate malformations, polydactyly, and kidney cysts. Mouse mutations in either Mks1 or B9d2 compromise ciliogenesis and result in phenotypes similar to those of MKS. Given the importance of these two B9 proteins to ciliogenesis, we examined the role of the third B9 protein, B9d1. Mice lacking B9d1 displayed polydactyly, kidney cysts, ductal plate malformations, and abnormal patterning of the neural tube, concomitant with compromised ciliogenesis, ciliary protein localization, and Hedgehog (Hh) signal transduction. These data prompted us to screen MKS patients for mutations in B9D1 and B9D2. We identified a homozygous c.301A>C (p.Ser101Arg) B9D2 mutation that segregates with MKS, affects an evolutionarily conserved residue, and is absent from controls. Unlike wild-type B9D2 mRNA, the p.Ser101Arg mutation failed to rescue zebrafish phenotypes induced by the suppression of b9d2. With coimmunoprecipitation and mass spectrometric analyses, we found that Mks1, B9d1, and B9d2 interact physically, but that the p.Ser101Arg mutation abrogates the ability of B9d2 to interact with Mks1, further suggesting that the mutation compromises B9d2 function. Our data indicate that B9d1 is required for normal Hh signaling, ciliogenesis, and ciliary protein localization and that B9d1 and B9d2 are essential components of a B9 protein complex, disruption of which causes MKS. PMID:21763481

Ciliary neurotrophic factor is an endogenous pyrogen.

Science.gov (United States)

Shapiro, L; Zhang, X X; Rupp, R G; Wolff, S M; Dinarello, C A

1993-09-15

Fever is initiated by the action of polypeptide cytokines called endogenous pyrogens, which are produced by the host during inflammation, trauma, or infection and which elevate the thermoregulatory set point in the hypothalamus. Ciliary neurotrophic factor (CNTF) supports the differentiation and survival of central and peripheral neurons. We describe the activity of CNTF as intrinsically pyrogenic in the rabbit. CNTF induced a monophasic fever which rose rapidly (within the first 12 min) following intravenous injection; CNTF fever was blocked by pretreatment with indomethacin. The fever induced by CNTF was not due to contaminating endotoxins. Increasing doses of CNTF resulted in prolongation of the fever, suggesting the subsequent induction of additional endogenous pyrogenic activity. After passive transfer of plasma obtained during CNTF-induced fever, endogenous pyrogen activity was not present in the circulation; CNTF also did not induce the endogenous pyrogens interleukin 1, tumor necrosis factor, or interleukin 6 in vitro. Nevertheless, a second endogenous pyrogen may originate within the central nervous system following the systemic injection of CNTF. Of the four endogenous pyrogens described to date (interleukin 1, tumor necrosis factor, interferon, and interleukin 6), CNTF, like interleukin 6, utilizes the cell-surface gp 130 signal-transduction apparatus.

[3H]acetylcholine synthesis in cultured ciliary ganglion neurons: effects of myotube membranes

International Nuclear Information System (INIS)

Gray, D.B.; Tuttle, J.B.

1987-01-01

Avian ciliary ganglion neurons in cell culture were examined for the capacity to synthesize acetylcholine (ACh) from the exogenously supplied precursor, choline. Relevant kinetic parameters of the ACh synthetic system in cultured neurons were found to be virtually the same as those of the ganglionic terminals in the intact iris. Neurons were cultured in the presence of and allowed to innervate pectoral muscle; this results in an capacity for ACh synthesis. In particular, the ability to increase ACh synthesis upon demand after stimulation is affected by interaction with the target. This effect is shown to be an acceleration of the maturation of the cultured neurons. Lysed and washed membrane remnants of the muscle target were able to duplicate, in part, this effect of live target tissue on neuronal transmitter metabolism. Culture medium conditioned by muscle, and by the membrane remnants of muscle, was without significant effect. Thus, substances secreted into the medium do not play a major role in this interaction. Neurons cultured with either muscle or muscle membrane remnants formed large, elongate structures on the target membrane surface. These were not seen in the absence of the target at the times examined. This morphological difference in terminal-like structures may parallel the developmental increases in size and vesicular content of ciliary ganglion nerve terminals in the chick iris, and may relate to the increased ACh synthetic activity. The results suggest that direct contact with an appropriate target membrane has a profound, retrograde influence upon neuronal metabolic and morphological maturation

Bio-inspired ciliary force sensor for robotic platforms

KAUST Repository

Ribeiro, Pedro; Khan, Mohammed Asadullah; Alfadhel, Ahmed; Kosel, Jü rgen; Franco, Fernando; Cardoso, Susana; Bernardino, Alexandre; Schmitz, Alexander; Santos-Victor, Jose; Jamone, Lorenzo

2017-01-01

The detection of small forces is of great interest in any robotic application that involves interaction with the environment (e.g., objects manipulation, physical human-robot interaction, minimally invasive surgery), since it allows the robot to detect the contacts early on and to act accordingly. In this letter, we present a sensor design inspired by the ciliary structure frequently found in nature, consisting of an array of permanently magnetized cylinders (cilia) patterned over a giant magnetoresistance sensor (GMR). When these cylinders are deformed in shape due to applied forces, the stray magnetic field variation will change the GMR sensor resistivity, thus enabling the electrical measurement of the applied force. In this letter, we present two 3 mm × 3 mm prototypes composed of an array of five cilia with 1 mm of height and 120 and 200 μm of diameter for each prototype. A minimum force of 333 μN was measured. A simulation model for determining the magnetized cylinders average stray magnetic field is also presented.

Bio-inspired ciliary force sensor for robotic platforms

KAUST Repository

Ribeiro, Pedro

2017-01-20

The detection of small forces is of great interest in any robotic application that involves interaction with the environment (e.g., objects manipulation, physical human-robot interaction, minimally invasive surgery), since it allows the robot to detect the contacts early on and to act accordingly. In this letter, we present a sensor design inspired by the ciliary structure frequently found in nature, consisting of an array of permanently magnetized cylinders (cilia) patterned over a giant magnetoresistance sensor (GMR). When these cylinders are deformed in shape due to applied forces, the stray magnetic field variation will change the GMR sensor resistivity, thus enabling the electrical measurement of the applied force. In this letter, we present two 3 mm × 3 mm prototypes composed of an array of five cilia with 1 mm of height and 120 and 200 μm of diameter for each prototype. A minimum force of 333 μN was measured. A simulation model for determining the magnetized cylinders average stray magnetic field is also presented.

Chloral hydrate alters the organization of the ciliary basal apparatus and cell organelles in sea urchin embryos

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Chakrabarti, A.; Schatten, H.; Mitchell, K. D.; Crosser, M.; Taylor, M.

1998-01-01

The mitotic inhibitor, chloral hydrate, induces ciliary loss in the early embryo phase of Lytechinus pictus. It causes a breakdown of cilia at the junction of the cilium and the basal body known as the basal plate. This leaves the plasma membrane temporarily unsealed. The basal apparatus accessory structures, consisting of the basal body, basal foot, basal foot cap, striated side arm, and striated rootlet, are either misaligned or disintegrated by treatment with chloral hydrate. Furthermore, microtubules which are associated with the basal apparatus are disassembled. Mitochondria accumulate at the base of cilia - underneath the plasma membrane - and show alterations in their structural organization. The accumulation of mitochondria is observed in 40% of all electron micrograph sections while 60% show the areas mostly devoid of mitochondria. The microvilli surrounding a cilium and striated rootlet remain intact in the presence of chloral hydrate. These results suggest that deciliation in early sea urchin embryos by chloral hydrate is caused by combined effects on the ciliary membrane and on microtubules in the cilia. Furthermore, it is suggested that chloral hydrate can serve as a tool to explore the cytoskeletal mechanisms that are involved in cilia motility in the developing sea urchin embryo.

Convergent evolution of RFX transcription factors and ciliary genes predated the origin of metazoans

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Chen Nansheng

2010-05-01

Full Text Available Abstract Background Intraflagellar transport (IFT genes, which are critical for the development and function of cilia and flagella in metazoans, are tightly regulated by the Regulatory Factor X (RFX transcription factors (TFs. However, how and when their evolutionary relationship was established remains unknown. Results We have identified evidence suggesting that RFX TFs and IFT genes evolved independently and their evolution converged before the first appearance of metazoans. Both ciliary genes and RFX TFs exist in all metazoans as well as some unicellular eukaryotes. However, while RFX TFs and IFT genes are found simultaneously in all sequenced metazoan genomes, RFX TFs do not co-exist with IFT genes in most pre-metazoans and thus do not regulate them in these organisms. For example, neither the budding yeast nor the fission yeast possesses cilia although both have well-defined RFX TFs. Conversely, most unicellular eukaryotes, including the green alga Chlamydomonas reinhardtii, have typical cilia and well conserved IFT genes but lack RFX TFs. Outside of metazoans, RFX TFs and IFT genes co-exist only in choanoflagellates including M. brevicollis, and only one fungus Allomyces macrogynus of the 51 sequenced fungus genomes. M. brevicollis has two putative RFX genes and a full complement of ciliary genes. Conclusions The evolution of RFX TFs and IFT genes were independent in pre-metazoans. We propose that their convergence in evolution, or the acquired transcriptional regulation of IFT genes by RFX TFs, played a pivotal role in the establishment of metazoan.

Aerobic fitness in children and young adults with primary ciliary dyskinesia

DEFF Research Database (Denmark)

Madsen, Astrid Hellerup; Green, Kent; Buchvald, Frederik

2013-01-01

BACKGROUND: Although aerobic fitness is regarded as an overall prognostic measure of morbidity and mortality, its evaluation in the chronic progressive sinopulmonary disease primary ciliary dyskinesia (PCD) has been infrequently and inconsistently reported. Here we assessed peak oxygen uptake (VO2...... multiple breath inert gas washout (N2 MBW) were assessed in a cross-sectional, single-occasion study of clinically stable children and young adults with PCD. We used a questionnaire including self-reported physical limitations in everyday life or in vigorous activities, and estimation of weekly hours...... patients. CONCLUSION: One-third of PCD patients exhibited substantially lower aerobic fitness than healthy subjects. Aerobic fitness correlated with FEV1, DLCO/VA and self-reported complaints of limitations in vigorous physical activity. These findings are most likely explained by PCD pulmonary disease...

Mucous flow and ciliary activity in the trachea of rats exposed to pulmonary irritant gas

Energy Technology Data Exchange (ETDEWEB)

Dalhamm, T; Rhodin, J

1956-01-01

Tracheal mucous secretion was increased in rats exposed to 10 ppM SO/sub 2/, 6 hr/day for 10 weeks. Mucous transport was decreased from 13.5 to 5 mm/min although ciliary beat remained a constant 1320 beats/min. The mucous layer was 5 times as thick as the normal ..mu..m. Histologically, cilia were generally unaffected whereas tracheal epithelium was irregular with deep crypts, and the lamina propria showed severe edema, fragmentation of collagen fibrils, and profuse vascularization with blood escaping perivascularly.

[Endoscopically controlled optimization of trans-scleral suture fixation of posterior chamber lenses in the ciliary sulcus].

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Althaus, C; Sundmacher, R

1993-08-01

Two technical difficulties have to be overcome in transscleral suture fixation of posterior chamber intraocular lenses (PCL) in the ciliary sulcus: first, exact needle penetration through the sulcus, and second, exact positioning of the PCL haptics in the sulcus. Incongruence of the two may lead to long-term complications by compression or even strangulation of ciliary processes. Intraocular endoscopy was used intraoperatively to visualize the site of needle penetration and the final location of the haptics in patients. It turned out that with our previously described standard techniques the precision was far less than anticipated. Thus, new technical ways had to be sought to improve the precision of positioning. In secondary implantation without perforating keratoplasty we achieved the best results when the needle was passed ab externo before opening the eye and before anterior vitrectomy, taking advantage of a precisely prepared sclerocorneal zone. Passing the needle ab externo in an already hypotonic eyeball gives much less precise results. In combination with perforating keratoplasty with an open-sky approach, needle penetration ab interno is reliable. Correct positioning of the PCL haptics is at least as difficult as correct needle penetration, a fact which up to now has mostly been ignored. In 33 consecutively operated eyes the technique of implantation and PCL design was varied under endoscopical control.(ABSTRACT TRUNCATED AT 250 WORDS)

The eye-catching cicada Hamza ciliaris (Linnaeus, 1758) comb. n. in Indonesia and the Pacific: taxonomie status, synonymy, and distribution (Homoptera, Cicadoidea)

NARCIS (Netherlands)

Duffels, J.P.

1991-01-01

The new combination Hamza ciliaris (Linnaeus) is proposed for a cicada species widely distributed in Maluku ( = Moluccas), Timor, Banda, Kei and Banggai Islands, the Philippines, and the Palau group of the Caroline Islands. The synonymy of five species, treated in the literature as junior synonyms

Studies on bicarbonate transporters and carbonic anhydrase in porcine non-pigmented ciliary epithelium

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Shahidullah, Mohammad; C-H, To; Pelis, Ryan M.; Delamere, Nicholas A

2009-01-01

Purpose Bicarbonate transport plays a role in aqueous humor (AH) secretion. Here, we examined bicarbonate transport mechanisms and carbonic anhydrase (CA) in porcine non-pigmented ciliary epithelium (NPE). Methods Cytoplasmic pH (pHi) was measured in cultured porcine NPE loaded with BCECF. Anion exchanger (AE), sodium bicarbonate cotransporter (NBC) and CA were examined by RT-PCR and immunolocalization. AH secretion was measured in the intact porcine eye using a fluorescein dilution technique. Results Anion exchanger AE2, CAII and CAIV were abundant in the NPE layer. In cultured NPE superfused with a CO2/HCO3− free HEPES buffer, exposure to a CO2/HCO3−-containing buffer caused a rapid acidification followed by a gradual pHi increase. Subsequent removal of CO2/HCO3− with HEPES buffer caused rapid alkalinization followed by gradual pHi decrease. The rate of gradual alkalinization after addition of HCO3−/CO2 was inhibited by sodium-free conditions, DIDS, CA inhibitors acetazolamide and methazolamide but not by Na-H exchange inhibitor dimethylamiloride or low chloride buffer. The phase of gradual acidification after removal of HCO3−/CO2 was inhibited by DIDS, acetazolamide, methazolamide and by low chloride buffer. DIDS reduced baseline pHi. In the intact eye, DIDS and acetazolamide reduced AH secretion by 25% and 44% respectively. Conclusion The results suggest the NPE uses a Na+-HCO3− cotransporter to import bicarbonate and a Cl−/HCO3− exchanger to export bicarbonate. CA influences the rate of bicarbonate transport. AE2, CAII and CAIV are enriched in the NPE layer of the ciliary body and their coordinated function may contribute to AH secretion by effecting bicarbonate transport into the eye. PMID:19011010

Control of ciliary motility by Ca2+: Integration of Ca2+-dependent functions and targets for Ca2+ action

International Nuclear Information System (INIS)

Evans, T.C.

1988-01-01

To identify functions that regulate Ca 2+ -induced ciliary reversal in Paramecium, mutants defective in terminating depolarization-induced backward swimming were selected. Six independent recessive mutations (k-shy) comprising two complementation groups, k-shyA and k-shyB, were identified. All mutants exhibited prolonged backward swimming in depolarizing solutions. Voltage clamp studies revealed that mutant Ca 2+ current amplitudes were reduced, but could be restored to wild type levels by EGTA injection. The recovery of the mutant Ca 2+ current from Ca 2+ -dependent inactivation, and the decay of the Ca 2+ -dependent K + and Ca 2+ -dependent Na + currents after depolarization were slow in k-shy compared to wild type. To identify protein targets of Ca 2+ action, ciliary proteins that interact with calmodulin (CaM) were characterized. With a 125 I-CaM blot assay, several CaM-binding proteins were identified including axonemal, soluble, and membrane-bound polypeptides. Competitive displacement studies with unlabeled Paramecium CaM, bovine CaM, and troponinC suggested that both protein types bind CaM with high affinity and specificity. To examine the presence of CaM-binding sites in intact axonemes, a filtration binding assay was developed

Hyperglycemia in Streptozotocin-Induced Diabetes Leads to Persistent Inflammation and Tissue Damage Following Uveitis Due to Reduced Levels of Ciliary Body Heme Oxygenase-1

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2006-01-01

Full Text Available This study investigated the heme oxygenase-1 (HO-1 and the endotoxin-induced uveitis (EIU in diabetic streptozotocin (STZ-hyperglycemic rats. STZ-hyperglycemic rats had impaired levels of the enzyme HO-1 within the ciliary bodies if compared with the nondiabetic rats. STZ-hyperglycemic rats also predisposed the eye to produce high levels of both the cytokines IL-1 β and CXCL8. Subsequent EIU further and significantly P<.01 increased the cytokines production, an effect partly prevented by hemin treatment. Most importantly, hemin, an inducer of heme oxygenase expression and activity, recovered the huge number of infiltrated polymorphonuclear leukocytes PMN within the ciliary bodies associated with STZ-hyperglycemic state and EIU damage. Impairment of the stress-sensitive enzyme HO-1 in STZ-hyperglycemic rats increases and prolongs the inflammatory response to EIU.

Induction of Functional 3D Ciliary Epithelium-Like Structure From Mouse Induced Pluripotent Stem Cells.

Science.gov (United States)

Kinoshita, Hirofumi; Suzuma, Kiyoshi; Kaneko, Jun; Mandai, Michiko; Kitaoka, Takashi; Takahashi, Masayo

2016-01-01

To generate ciliary epithelium (CE) from mouse induced pluripotent stem (iPS) cells. Recently, a protocol for self-organizing optic cup morphogenesis in three-dimensional culture was reported, and it was suggested that ocular tissue derived from neural ectoderm could be differentiated. We demonstrated that a CE-like double-layered structure could be induced in simple culture by using a modified Eiraku differentiation protocol. Differentiation of a CE-like double-layered structure could be promoted by glycogen synthase kinase 3β (GSK-3β) inhibitor. Connexin43 and aquaporin1 were expressed in both thin layers, and induced CE-like cells expressed ciliary marker genes, such as cyclinD2, zic1, tgfb2, aldh1a3, wfdc1, otx1, BMP4, and BMP7. Increases in cytoplasmic and nuclear β-catenin in aggregates of the CE-like double-layered structure were confirmed by Western blot analysis. In addition, tankyrase inhibitor prevented the induction of the CE-like double-layered structure by GSK-3β inhibitor. Dye movement from pigmented cells to nonpigmented cells in the mouse iPS cell-derived CE-like structure was observed in a fluid movement experiment, consistent with the physiological function of CE in vivo. We could differentiate CE from mouse iPS cells in the present study. In the future, we hope that this CE-like complex will become useful as a graft for transplantation therapy in pathologic ocular hypotension due to CE dysfunction, and as a screening tool for the development of drugs for diseases associated with CE function.

Melatonin synthesis in the human ciliary body triggered by TRPV4 activation: Involvement of AANAT phosphorylation.

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Alkozi, Hanan Awad; Perez de Lara, María J; Pintor, Jesús

2017-09-01

Melatonin is a substance synthesized in the pineal gland as well as in other organs. This substance is involved in many ocular functions, giving its synthesis in numerous eye structures. Melatonin is synthesized from serotonin through two enzymes, the first limiting step into the synthesis of melatonin being aralkylamine N-acetyltransferase (AANAT). In this current study, AANAT phosphorylation after the activation of TRPV4 was studied using human non-pigmented epithelial ciliary body cells. Firstly, it was necessary to determine the adequate time and dose of the TRPV4 agonist GSK1016790A to reach the maximal phosphorylation of AANAT. An increase of 72% was observed after 5 min incubation with 10 nM GSK (**p melatonin synthesis. The involvement of a TRPV4 channel in melatonin synthesis was verified by antagonist and siRNA studies as a previous step to studying intracellular signalling. Studies performed on the second messengers involved in GSK induced AANAT phosphorylation were carried out by inhibiting several pathways. In conclusion, the activation of calmodulin and calmodulin-dependent protein kinase II was confirmed, as shown by the cascade seen in AANAT phosphorylation (***p melatonin levels. In conclusion, the activation of a TRPV4 present in human ciliary body epithelial cells produced an increase in AANAT phosphorylation and a further melatonin increase by a mechanism in which Ca-calmodulin and the calmodulin-dependent protein kinase II are involved. Copyright © 2017 Elsevier Ltd. All rights reserved.

Hearing loss in children with primary ciliary dyskinesia.

Science.gov (United States)

Kreicher, Kathryn L; Schopper, Heather K; Naik, Akash N; Hatch, Jonathan L; Meyer, Ted A

2018-01-01

To evaluate the type and severity of hearing impairment in pediatric patients with primary ciliary dyskinesia (PCD) and relate these measures to patient demographics, treatment options, and other otologic factors. A retrospective analysis of children with a diagnosis of PCD, Kartagener's syndrome, or situs inversus in the AudGen Database was conducted. Audiograms were analyzed for type of hearing loss (HL), severity, laterality, and progression. Medical charts were reviewed to identify factors that influence severity and progression of hearing loss. 56 patients met inclusion criteria and 42 patients had HL. 66.6% had bilateral and 33.3% had unilateral loss (70 total ears with HL). Conductive hearing loss (CHL) was the most common type of HL, though 30% of children had some sensorineural component to their hearing loss. 92.9% of children with HL received at least one diagnosis of otitis media, but HL did not improve in the majority (77.8%) of ears in our study regardless of ear tube placement. Slight to mild CHL and all types of otitis media are prevalent among patients with PCD, and some of these children have sensorineural hearing loss (SNHL). All patients diagnosed with situs inversus at birth should be evaluated by an otolaryngologist. Copyright © 2017 Elsevier B.V. All rights reserved.

IFT20 modulates ciliary PDGFRα signaling by regulating the stability of Cbl E3 ubiquitin ligases

DEFF Research Database (Denmark)

Schmid, Fabian Marc; Schou, Kenneth Bødtker; Vilhelm, Martin Juel

2018-01-01

ciliogenesis, and ciliary localization of the receptor is required for its appropriate ligand-mediated activation by PDGF-AA. However, the mechanisms regulating sorting of PDGFRα and feedback inhibition of PDGFRα signaling at the cilium are unknown. Here, we provide evidence that intraflagellar transport...... protein 20 (IFT20) interacts with E3 ubiquitin ligases c-Cbl and Cbl-b and is required for Cbl-mediated ubiquitination and internalization of PDGFRα for feedback inhibition of receptor signaling. In wild-type cells treated with PDGF-AA, c-Cbl becomes enriched in the cilium, and the receptor...

CCDC39 is required for assembly of inner dynein arms and the dynein regulatory complex and for normal ciliary motility in humans and dogs

DEFF Research Database (Denmark)

Merveille, Anne-Christine; Davis, Erica E; Becker-Heck, Anita

2011-01-01

Primary ciliary dyskinesia (PCD) is an inherited disorder characterized by recurrent infections of the upper and lower respiratory tract, reduced fertility in males and situs inversus in about 50% of affected individuals (Kartagener syndrome). It is caused by motility defects in the respiratory c...

Safety assessment of thiolated polymers: effect on ciliary beat frequency in human nasal epithelial cells.

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Palmberger, Thomas F; Augustijns, Patrick; Vetter, Anja; Bernkop-Schnürch, Andreas

2011-12-01

The aim of this study was to investigate the nasal safety of gel formulations of thiolated polymers (thiomers) by assessing their effect on ciliary beat frequency (CBF) in human nasal epithelial cells. Poly(acrylic acid) 450 kDa-cysteine (PAA-cys) and alginate-cysteine (alg-cys) were synthesized by covalent attachment of L-cysteine to the polymeric backbone. The cationic polymer chitosan-thiobutylamidine (chito-TBA) was synthesized by attaching iminothiolane to chitosan. CBF using was measured by a photometric system. CBF was measured before incubating the cells with test gels, during incubation and after washing out the polymeric test gels to evaluate reversibility of cilio-inhibition. The influence of viscosity on CBF was determined by using hydroxyethylcellulose (HEC)-gels of various concentrations. Ciliary beating was observed to be affected by viscosity, but cilia were still beating in the presence of a HEC-gel displaying an apparent viscosity of 25 Pa.s. In case of thiolated polymers and their unmodified control, a concentration-dependent decrease in CBF could be observed. PAA-cys, alg-cys, chito-TBA and their corresponding unmodified controls exhibited a moderate cilio-inhibitory effect, followed by a partial recovery of CBF when used at a concentration of 1%. Alg-cys 2% and chito-TBA 2% (m/v) gels exhibited severe cilio-inhibition, which was partially reversible. L-cysteine and reduced glutathione led to mild cilio-inhibition at concentrations of 3% (m/v). Taking into account that dilution after application and cilio-modifying effects is usually more pronounced under in vitro conditions, thiomers can be considered as suitable excipients for nasal drug delivery systems.

Proteomic analysis of isolated chlamydomonas centrioles reveals orthologs of ciliary-disease genes.

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Keller, Lani C; Romijn, Edwin P; Zamora, Ivan; Yates, John R; Marshall, Wallace F

2005-06-21

The centriole is one of the most enigmatic organelles in the cell. Centrioles are cylindrical, microtubule-based barrels found in the core of the centrosome. Centrioles also act as basal bodies during interphase to nucleate the assembly of cilia and flagella. There are currently only a handful of known centriole proteins. We used mass-spectrometry-based MudPIT (multidimensional protein identification technology) to identify the protein composition of basal bodies (centrioles) isolated from the green alga Chlamydomonas reinhardtii. This analysis detected the majority of known centriole proteins, including centrin, epsilon tubulin, and the cartwheel protein BLD10p. By combining proteomic data with information about gene expression and comparative genomics, we identified 45 cross-validated centriole candidate proteins in two classes. Members of the first class of proteins (BUG1-BUG27) are encoded by genes whose expression correlates with flagellar assembly and which therefore may play a role in ciliogenesis-related functions of basal bodies. Members of the second class (POC1-POC18) are implicated by comparative-genomics and -proteomics studies to be conserved components of the centriole. We confirmed centriolar localization for the human homologs of four candidate proteins. Three of the cross-validated centriole candidate proteins are encoded by orthologs of genes (OFD1, NPHP-4, and PACRG) implicated in mammalian ciliary function and disease, suggesting that oral-facial-digital syndrome and nephronophthisis may involve a dysfunction of centrioles and/or basal bodies. By analyzing isolated Chlamydomonas basal bodies, we have been able to obtain the first reported proteomic analysis of the centriole.

Long-term follow-up of patients with retinitis pigmentosa (RP) receiving intraocular ciliary neurotrophic factor implants

Science.gov (United States)

Birch, David G.; Bennett, Lea D.; Duncan, Jacque L.; Weleber, Richard G.; Pennesi, Mark E.

2016-01-01

Purpose To evaluate the long-term efficacy of ciliary neurotrophic factor delivered via an intraocular encapsulated cell implant for the treatment of retinitis pigmentosa (RP). Design Long-term follow up of a multicenter, sham-controlled study. Methods Thirty-six patients at three CNTF4 sites were randomly assigned to receive a high- or low- dose implant in one eye and sham surgery in the fellow eye. The primary endpoint (change in visual field sensitivity at 12 months) has been reported previously.1 Here we report long-term visual acuity, visual field and optical coherence tomography (OCT) outcomes in 24 patients either retaining or explanting the device at 24 months relative to sham-treated eyes. Results Eyes retaining the implant showed significantly greater visual field loss from baseline than either explanted eyes or sham eyes through 42 months. By 60 months and continuing through 96 months, visual field loss was comparable among sham-treated eyes, eyes retaining the implant and explanted eyes, as was visual acuity and OCT macular volume. Conclusions Over the short term, ciliary neurotrophic factor released continuously from an intra-vitreal implant lead to loss of total visual field sensitivity that was greater than the natural progression in the sham-treated eye. This additional loss of sensitivity related to the active implant was reversible when the implant was removed. Over the long term (60 – 96 months), there was no evidence of efficacy for visual acuity, visual field sensitivity or OCT measures of retinal structure. PMID:27457255

Mutations in ZMYND10, a gene essential for proper axonemal assembly of inner and outer dynein arms in humans and flies, cause primary ciliary dyskinesia

DEFF Research Database (Denmark)

Moore, Daniel J; Onoufriadis, Alexandros; Shoemark, Amelia

2013-01-01

Primary ciliary dyskinesia (PCD) is a ciliopathy characterized by airway disease, infertility, and laterality defects, often caused by dual loss of the inner dynein arms (IDAs) and outer dynein arms (ODAs), which power cilia and flagella beating. Using whole-exome and candidate-gene Sanger resequ...

Localization of multidrug resistance-associated protein 2 in the nonpigmented ciliary epithelium of the eye.

Science.gov (United States)

Pelis, Ryan M; Shahidullah, Mohammad; Ghosh, Sikha; Coca-Prados, Miguel; Wright, Stephen H; Delamere, Nicholas A

2009-05-01

The nonpigmented epithelium (NPE) of the ciliary body represents an important component of the blood-aqueous barrier of the eye. Many therapeutic drugs penetrate poorly across the NPE into the aqueous humor of the eye interior. Several of these therapeutic drugs, such as methotrexate, vincristine, and etoposide, are substrates of the multidrug resistance-associated protein 2 (MRP2). Abundant MRP2 protein was detected by Western blot in homogenates of human ciliary body and freshly dissected porcine NPE. In cultured porcine NPE, the intracellular accumulation of the MRP2 substrates calcein (1.8-fold), 5-(and-6)-carboxy-2',7'-dichlorofluorescein (22.1-fold), and doxorubicin (1.9-fold) was significantly increased in the presence of 50 microM MK571 ((E)-3-[[[3-[2-(7-chloro-2-quinolinyl)-ethenyl]phenyl]-[[3-dimethylamino)-3-oxopropyl]thio]methyl]thio]-propanoic acid), an MRP inhibitor. In addition, the intracellular accumulation of the MRP2 substrate glutathione methylfluorescein was increased by 50 microM MK571 (4.3-fold), 500 microM indomethacin (2.6-fold), and 50 microM cyclosporin A (2.1-fold) but not by 500 microM sulfinpyrazone. These data are consistent with MRP2-mediated transport activity in cultured NPE, and MRP2 mRNA (reverse transcriptase-polymerase chain reaction) and protein (Western blot) were detected in the cultured cells. Immunolocalization studies in native human and porcine eyes showed MRP2 protein at the apical interface of the NPE and pigmented cell layers. Close examination of MRP2 immunoreactivity supported the conclusion that MRP2 is localized in the apical membrane of the NPE. MRP2 at the apical membrane of NPE cells may be involved in protecting intraocular tissues from exposure to potentially harmful toxins.

The ciliary margin zone of the mammalian retina generates retinal ganglion cells

Science.gov (United States)

Marcucci, Florencia; Murcia-Belmonte, Veronica; Coca, Yaiza; Ferreiro-Galve, Susana; Wang, Qing; Kuwajima, Takaaki; Khalid, Sania; Ross, M. Elizabeth; Herrera, Eloisa; Mason, Carol

2016-01-01

Summary The retina of lower vertebrates grows continuously by integrating new neurons generated from progenitors in the ciliary margin zone (CMZ). Whether the mammalian CMZ provides the neural retina with retinal cells is controversial. Live-imaging of embryonic retina expressing eGFP in the CMZ shows that cells migrate laterally from the CMZ to the neural retina where differentiated retinal ganglion cells (RGCs) reside. As Cyclin D2, a cell-cycle regulator, is enriched in ventral CMZ, we analyzed Cyclin D2−/− mice to test whether the CMZ is a source of retinal cells. Neurogenesis is diminished in Cyclin D2 mutants, leading to a reduction of RGCs in the ventral retina. In line with these findings, in the albino retina, the decreased production of ipsilateral RGCs is correlated with fewer Cyclin D2+ cells. Together, these results implicate the mammalian CMZ as a neurogenic site that produces RGCs and whose proper generation depends on Cyclin D2 activity. PMID:28009286

The Meckel syndrome- associated protein MKS1 functionally interacts with components of the BBSome and IFT complexes to mediate ciliary trafficking and hedgehog signaling

Science.gov (United States)

Barrington, Chloe L.; Katsanis, Nicholas

2017-01-01

The importance of primary cilia in human health is underscored by the link between ciliary dysfunction and a group of primarily recessive genetic disorders with overlapping clinical features, now known as ciliopathies. Many of the proteins encoded by ciliopathy-associated genes are components of a handful of multi-protein complexes important for the transport of cargo to the basal body and/or into the cilium. A key question is whether different complexes cooperate in cilia formation, and whether they participate in cilium assembly in conjunction with intraflagellar transport (IFT) proteins. To examine how ciliopathy protein complexes might function together, we have analyzed double mutants of an allele of the Meckel syndrome (MKS) complex protein MKS1 and the BBSome protein BBS4. We find that Mks1; Bbs4 double mutant mouse embryos exhibit exacerbated defects in Hedgehog (Hh) dependent patterning compared to either single mutant, and die by E14.5. Cells from double mutant embryos exhibit a defect in the trafficking of ARL13B, a ciliary membrane protein, resulting in disrupted ciliary structure and signaling. We also examined the relationship between the MKS complex and IFT proteins by analyzing double mutant between Mks1 and a hypomorphic allele of the IFTB component Ift172. Despite each single mutant surviving until around birth, Mks1; Ift172avc1 double mutants die at mid-gestation, and exhibit a dramatic failure of cilia formation. We also find that Mks1 interacts genetically with an allele of Dync2h1, the IFT retrograde motor. Thus, we have demonstrated that the MKS transition zone complex cooperates with the BBSome to mediate trafficking of specific trans-membrane receptors to the cilium. Moreover, the genetic interaction of Mks1 with components of IFT machinery suggests that the transition zone complex facilitates IFT to promote cilium assembly and structure. PMID:28291807

The Meckel syndrome- associated protein MKS1 functionally interacts with components of the BBSome and IFT complexes to mediate ciliary trafficking and hedgehog signaling.

Directory of Open Access Journals (Sweden)

Sarah C Goetz

Full Text Available The importance of primary cilia in human health is underscored by the link between ciliary dysfunction and a group of primarily recessive genetic disorders with overlapping clinical features, now known as ciliopathies. Many of the proteins encoded by ciliopathy-associated genes are components of a handful of multi-protein complexes important for the transport of cargo to the basal body and/or into the cilium. A key question is whether different complexes cooperate in cilia formation, and whether they participate in cilium assembly in conjunction with intraflagellar transport (IFT proteins. To examine how ciliopathy protein complexes might function together, we have analyzed double mutants of an allele of the Meckel syndrome (MKS complex protein MKS1 and the BBSome protein BBS4. We find that Mks1; Bbs4 double mutant mouse embryos exhibit exacerbated defects in Hedgehog (Hh dependent patterning compared to either single mutant, and die by E14.5. Cells from double mutant embryos exhibit a defect in the trafficking of ARL13B, a ciliary membrane protein, resulting in disrupted ciliary structure and signaling. We also examined the relationship between the MKS complex and IFT proteins by analyzing double mutant between Mks1 and a hypomorphic allele of the IFTB component Ift172. Despite each single mutant surviving until around birth, Mks1; Ift172avc1 double mutants die at mid-gestation, and exhibit a dramatic failure of cilia formation. We also find that Mks1 interacts genetically with an allele of Dync2h1, the IFT retrograde motor. Thus, we have demonstrated that the MKS transition zone complex cooperates with the BBSome to mediate trafficking of specific trans-membrane receptors to the cilium. Moreover, the genetic interaction of Mks1 with components of IFT machinery suggests that the transition zone complex facilitates IFT to promote cilium assembly and structure.

OFD1, as a Ciliary Protein, Exhibits Neuroprotective Function in Photoreceptor Degeneration Models.

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Juan Wang

Full Text Available Ofd1 is a newly identified causative gene for Retinitis pigmentosa (RP, a photoreceptor degenerative disease. This study aimed to examine Ofd1 localization in retina and further to investigate its function in photoreceptor degeneration models. Ofd1 localization in rat retina was examined using immunofluorescence. N-methyl-N-nitrosourea (MNU-induced rats and Royal College of Surgeons (RCS rats were used as photoreceptor degeneration models. The expression pattern of Ofd1, other ciliary associated genes and Wnt signaling pathway genes were examined in rat models. Furthermore, pEGFP-Ofd1-CDS and pSUPER-Ofd1-shRNA were constructed to overexpress and knockdown the expression level in 661W and R28 cells. MNU was also used to induce cell death. Cilia formation was observed using immunocytochemistry (ICC. Reactive oxygen species (ROS were detected using the 2', 7'-Dichlorofluorescin diacetate (DCFH-DA assay. Apoptosis genes expression was examined using qRT-PCR, Western blotting and fluorescence-activated cell sorting (FACS. Ofd1 localized to outer segments of rat retina photoreceptors. Ofd1 and other ciliary proteins expression levels increased from the 1st and 4th postnatal weeks and decreased until the 6th week in the RCS rats, while their expression consistently decreased from the 1st and 7th day in the MNU rats. Moreover, Wnt signaling pathway proteins expression was significantly up-regulated in both rat models. Knockdown of Ofd1 expression resulted in a smaller population, shorter length of cell cilia, and lower cell viability. Ofd1 overexpression partially attenuated MNU toxic effects by reducing ROS levels and mitigating apoptosis. To the best of our knowledge, this is the first study demonstrating Ofd1 localization and its function in rat retina and in retinal degeneration rat models. Ofd1 plays a role in controlling photoreceptor cilium length and number. Importantly, it demonstrates a neuroprotective function by protecting the photoreceptor

The development of the larval nervous system, musculature and ciliary bands of Pomatoceros lamarckii (Annelida: heterochrony in polychaetes

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Shimeld Sebastian M

2006-10-01

Full Text Available Abstract Background To understand the evolution of animals it is essential to have taxon sampling across a representative spread of the animal kingdom. With the recent rearrangement of most of the Bilateria into three major clades (Ecdysozoa, Lophotrochozoa and Deuterostomia it has become clear that the Lophotrochozoa are relatively poorly represented in our knowledge of animal development, compared to the Ecdysozoa and Deuterostomia. We aim to contribute towards redressing this balance with data on the development of the muscular, nervous and ciliary systems of the annelid Pomatoceros lamarckii (Serpulidae. We compare our data with other lophotrochozoans. Results P. lamarckii develops locomotory and feeding structures that enable it to become a swimming, planktotrophic larva within 24 hours. Formation of the trochophore includes development of a prototroch, metatroch and neurotroch, development of apical and posterior nervous elements at similar times, and development of musculature around the ciliary bands and digestive tract prior to development of any body wall muscles. The adult nervous and muscular systems are essentially preformed in the late larva. Interestingly, the muscular systems of the larvae and juvenile worms do not include the circular muscles of the body wall, which are considered to be plesiomorphic for annelids, although the possibility that circular muscles develop after these stages cannot be ruled out at this point. Conclusion A comparison between polychaetes shows variability in the timing (heterochrony of development of body wall muscles and elements of the nervous system. These heterochronies are one route for evolution of different life history strategies, such as adaptations to feeding requirements.

Acute versus chronic loss of mammalian Azi1/Cep131 results in distinct ciliary phenotypes.

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Emma A Hall

Full Text Available Defects in cilium and centrosome function result in a spectrum of clinically-related disorders, known as ciliopathies. However, the complex molecular composition of these structures confounds functional dissection of what any individual gene product is doing under normal and disease conditions. As part of an siRNA screen for genes involved in mammalian ciliogenesis, we and others have identified the conserved centrosomal protein Azi1/Cep131 as required for cilia formation, supporting previous Danio rerio and Drosophila melanogaster mutant studies. Acute loss of Azi1 by knock-down in mouse fibroblasts leads to a robust reduction in ciliogenesis, which we rescue by expressing siRNA-resistant Azi1-GFP. Localisation studies show Azi1 localises to centriolar satellites, and traffics along microtubules becoming enriched around the basal body. Azi1 also localises to the transition zone, a structure important for regulating traffic into the ciliary compartment. To study the requirement of Azi1 during development and tissue homeostasis, Azi1 null mice were generated (Azi1(Gt/Gt. Surprisingly, Azi1(Gt/Gt MEFs have no discernible ciliary phenotype and moreover are resistant to Azi1 siRNA knock-down, demonstrating that a compensation mechanism exists to allow ciliogenesis to proceed despite the lack of Azi1. Cilia throughout Azi1 null mice are functionally normal, as embryonic patterning and adult homeostasis are grossly unaffected. However, in the highly specialised sperm flagella, the loss of Azi1 is not compensated, leading to striking microtubule-based trafficking defects in both the manchette and the flagella, resulting in male infertility. Our analysis of Azi1 knock-down (acute loss versus gene deletion (chronic loss suggests that Azi1 plays a conserved, but non-essential trafficking role in ciliogenesis. Importantly, our in vivo analysis reveals Azi1 mediates novel trafficking functions necessary for flagellogenesis. Our study highlights the

Exogenous ciliary neurotrophic factor (CNTF) reduces synaptic depression during repetitive stimulation.

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Garcia, Neus; Santafé, Manel M; Tomàs, Marta; Priego, Mercedes; Obis, Teresa; Lanuza, Maria A; Besalduch, Nuria; Tomàs, Josep

2012-09-01

It has been shown that ciliary neurotrophic factor (CNTF) has trophic and maintenance effects on several types of peripheral and central neurons, glia, and cells outside the nervous system. Both CNTF and its receptor, CNTF-Rα, are expressed in the muscle. We use confocal immunocytochemistry to show that the trophic cytokine and its receptor are present in the pre- and post-synaptic sites of the neuromuscular junctions (NMJs). Applied CNTF (7.5-200 ng/ml, 60 min-3 h) does not acutely affect spontaneous potentials (size or frequency) or quantal content of the evoked acetylcholine release from post-natal (in weak or strong axonal inputs on dually innervated end plates or in the most mature singly innervated synapses at P6) or adult (P30) NMJ of Levator auris longus muscle of the mice. However, CNTF reduces roughly 50% the depression produced by repetitive stimulation (40 Hz, 2 min) on the adult NMJs. Our findings indicate that, unlike neurotrophins, exogenous CNTF does not acutely modulate transmitter release locally at the mammalian neuromuscular synapse but can protect mature end plates from activity-induced synaptic depression. © 2012 Peripheral Nerve Society.

Cell context-specific expression of primary cilia in the human testis and ciliary coordination of Hedgehog signalling in mouse Leydig cells

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Berg Nygaard, Marie; Almstrup, Kristian; Lindbæk, Louise

2015-01-01

Primary cilia are sensory organelles that coordinate numerous cellular signalling pathways during development and adulthood. Defects in ciliary assembly or function lead to a series of developmental disorders and diseases commonly referred to as ciliopathies. Still, little is known about...... cells of mature seminiferous epithelium, but present in Sertoli cell-only tubules in Klinefelter syndrome testis. Peritubular cells in atrophic testis produce overly long cilia. Furthermore cultures of growth-arrested immature mouse Leydig cells express primary cilia that are enriched in components...

Pathogenesis of Congenital Rubella Virus Infection in Human Fetuses: Viral Infection in the Ciliary Body Could Play an Important Role in Cataractogenesis

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Thong Van Nguyen

2015-01-01

Interpretation: Our study based on the pathological examination demonstrated that the rubella virus infection occurred via systemic organs of human fetuses. This fact was confirmed by immunohistochemistry and direct detection of viral RNA in multiple organs. To the best of our knowledge, this study is the first report demonstrating that the rubella virus infection occurred via systemic organs of the human body. Importantly, virus infection of the ciliary body could play an important role in cataractogenesis.

Up-regulation of Ciliary Neurotrophic Factor in Astrocytes by Aspirin

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Modi, Khushbu K.; Sendtner, Michael; Pahan, Kalipada

2013-01-01

Ciliary neurotrophic factor (CNTF) is a promyelinating trophic factor, and the mechanisms by which CNTF expression could be increased in the brain are poorly understood. Acetylsalicylic acid (aspirin) is one of the most widely used analgesics. Interestingly, aspirin increased mRNA and protein expression of CNTF in primary mouse and human astrocytes in a dose- and time-dependent manner. Aspirin induced the activation of protein kinase A (PKA) but not protein kinase C (PKC). H-89, an inhibitor of PKA, abrogated aspirin-induced expression of CNTF. The activation of cAMP-response element-binding protein (CREB), but not NF-κB, by aspirin, the abrogation of aspirin-induced expression of CNTF by siRNA knockdown of CREB, the presence of a consensus cAMP-response element in the promoter of CNTF, and the recruitment of CREB and CREB-binding protein to the CNTF promoter by aspirin suggest that aspirin increases the expression of the Cntf gene via the activation of CREB. Furthermore, we demonstrate that aspirin-induced astroglial CNTF was also functionally active and that supernatants of aspirin-treated astrocytes of wild type, but not Cntf null, mice increased myelin-associated proteins in oligodendrocytes and protected oligodendrocytes from TNF-α insult. These results highlight a new and novel myelinogenic property of aspirin, which may be of benefit for multiple sclerosis and other demyelinating disorders. PMID:23653362

No evidence of cholesteatoma in untreated otitis media with effusion in children with primary ciliary dyskinesia.

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Ghedia, Reshma; Ahmed, Jahangir; Navaratnam, Annakan; Harcourt, Jonny

2018-02-01

Primary Ciliary Dyskinesia (PCD) describes a group of inherited disorders that result in abnormal ciliary motion leading to mucous stasis. Clinical features include almost universally otitis media with effusion (OME), particularly in infants. PCD patients provide us with a cohort of patients with OME that is not treated with ventilatory tube (VT) insertion as these have been shown to result in frequent complications including chronic otorrhoea, early extrusion and persistent perforation without significant improvement to hearing in the long term. This cohort was used to investigate whether children with PCD and OME not treated with VT were predisposed to cholesteatoma formation in the setting of a paediatric quaternary referral centre. A retrospective chart review was performed of all the children attending a multi-disciplinary PCD clinic at a national quaternary referral centre with a diagnosis of OME. We reviewed otoscopic findings, and audiometry and tympanometry results. We assessed the children in four groups: Watchful waiting, hearing aids, VT, and VT and hearing aids. One-hundred-and-one of 107 patients included in the study had a diagnosis of otitis media with effusion. No child with OME and PCD was diagnosed with a cholesteatoma during the follow up period. The only children who had insertion of a ventilatory tube were those who had the procedure prior to the formal diagnosis of PCD. We found a significant complication rate in the children with VT insertion. Hearing improved over time. The prevalence of retraction pockets in untreated OME was 1.72% (3 out of 174 ears). In children with PCD, OME is an almost universal finding in younger children, but not in adolescents. The study supports the current preference to avoid VT insertion in children with PCD as it confers a significantly higher rate of complications. No cases of cholesteatoma were found in this cohort of PCD children with OME managed without VTs. Crown Copyright © 2017. Published by Elsevier B

Immunohistochemical and ultrastructural properties of the larval ciliary band-associated strand in the sea urchin Hemicentrotus pulcherrimus.

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Katow, Hideki; Katow, Tomoko; Yoshida, Hiromi; Kiyomoto, Masato; Uemura, Isao

2016-01-01

The swimming activity of sea urchin larvae is dependent on the ciliary band (CB) on the larval surface and is regulated by several neurotransmitters, including serotonin (5HT), dopamine, and γ-aminobutyric acid (GABA). However, the CB signal transmission mechanism remains unknown. The present study investigated the structural relationship between the CB and external signal receptors by immunohistochemical and transmission electron microscopic analyses of sea urchin, Hemicentrotus pulcherrimus, larvae. Glutamate decarboxylase (GAD; GABA synthetase) was detected in a strand of multiple cells along the circumoral CB in 6-arm plutei. The GAD-expressing strand was closely associated with the CB on the oral ectoderm side. The ciliary band-associated strand (CBAS) also expressed the 5HT receptor (5HThpr) and encephalopsin (ECPN) throughout the cytoplasm and comprised 1- to 2-μm diameter axon-like long stretched regions and sporadic 6- to 7-μm diameter bulbous nucleated regions (perikarya) that protruded into the oral ectoderm side. Besides the laterally polarized morphology of the CBAS cells, Epith-2, which is the epithelial lateral cell surface-specific protein of the sea urchin embryo and larva, was expressed exclusively by perikarya but not by the axon-like regions. The CBAS exposed its narrow apical surface on the larval epithelium between the CB and squamous cells and formed adherens junctions (AJs) on the apical side between them. Despite the presence of the CBAS axon-like regions, tubulins, such as α-, β-, and acetylated α-tubulins, were not detected. However, the neuroendocrine cell marker protein synaptophysin was detected in the axon-like regions and in bouton-like protrusions that contained numerous small ultrastructural vesicles. The unique morphology of the CBAS in the sea urchin larva epithelium had not been reported. The CBAS expresses a remarkable number of receptors to environmental stimuli and proteins that are probably involved in signal

Reduced anaerobic and aerobic performance in children with primary ciliary dyskinesia.

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Simsek, Senem; Inal-Ince, Deniz; Cakmak, Aslihan; Emiralioglu, Nagehan; Calik-Kutukcu, Ebru; Saglam, Melda; Vardar-Yagli, Naciye; Ozcelik, Hayriye Ugur; Sonbahar-Ulu, Hazal; Bozdemir-Ozel, Cemile; Kiper, Nural; Arikan, Hulya

2018-05-01

Primary ciliary dyskinesia (PCD) restricts lifestyle and increases morbidity. The aim of the study was to investigate anaerobic and aerobic performance in children with PCD and their healthy counterparts. Thirty-one children with PCD and 29 age- and sex-matched healthy subjects were studied. Pulmonary function, hand grip strength (HGS), quadriceps strength (QMS), physical activity, anaerobic capacity (muscle power sprint test), and aerobic performance (modified shuttle walk test (MSWT)) were determined. Pulmonary function, HGS, QMS, mean anaerobic power (MAP), and MSWT distance in PCD were significantly lower than those of healthy subjects (p aerobic performance is impaired in PCD from the early stages. Age determines anaerobic performance. Gender is the determinant of aerobic performance. Whether skeletal muscle characteristics and sex-related changes in body composition affect anaerobic and aerobic capacity in PCD children warrants further study. What is Known: • Exercise performance is determined by anaerobic and aerobic power. • Few studies have shown that PCD patients have lower aerobic performance which is associated with impaired lung function. What is New: • The present research indicated that both anaerobic and aerobic exercise capacity determined using field testing is impaired in PCD from the early stages. • Anaerobic capacity was found to be independently associated with age in PCD. Higher aerobic performance is independently associated with male gender.

Intraocular gene transfer of ciliary neurotrophic factor rescues photoreceptor degeneration in RCS rats.

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Huang, Shun-Ping; Lin, Po-Kang; Liu, Jorn-Hon; Khor, Chin-Ni; Lee, Yih-Jing

2004-01-01

Ciliary neurotrophic factor (CNTF) is known as an important factor in the regulation of retinal cell growth. We used both recombinant CNTF and an adenovirus carrying the CNTF gene to regulate retinal photoreceptor expression in a retinal degenerative animal, Royal College of Surgeons (RCS) rats. Cells in the outer nuclear layer of the retinae from recombinant-CNTF-treated, adenoviral-CNTF-treated, saline-operated, and contralateral untreated preparations were examined for those exhibiting CNTF photoreceptor protective effects. Cell apoptosis in the outer nuclear layer of the retinae was also detected. It was found that CNTF had a potent effect on delaying the photoreceptor degeneration process in RCS rats. Furthermore, adenovirus CNTF gene transfer was proven to be better at rescuing photoreceptors than that when using recombinant CNTF, since adenoviral CNTF prolonged the photoreceptor protection effect. The function of the photoreceptors was also examined by taking electroretinograms of different animals. Adenoviral-CNTF-treated eyes showed better retinal function than did the contralateral control eyes. This study indicates that adenoviral CNTF effectively rescues degenerating photoreceptors in RCS rats. Copyright 2004 National Science Council, ROC and S. Karger AG, Basel

STOCHASTIC SIMULATION FOR BUFFELGRASS (Cenchrus ciliaris L. PASTURES IN MARIN, N. L., MEXICO

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José Romualdo Martínez-López

2014-04-01

Full Text Available A stochastic simulation model was constructed to determine the response of net primary production of buffelgrass (Cenchrus ciliaris L. and its dry matter intake by cattle, in Marín, NL, México. Buffelgrass is very important for extensive livestock industry in arid and semiarid areas of northeastern Mexico. To evaluate the behavior of the model by comparing the model results with those reported in the literature was the objective in this experiment. Model simulates the monthly production of dry matter of green grass, as well as its conversion to senescence and dry grass and eventually to mulch, depending on precipitation and temperature. Model also simulates consumption of green and dry grass for cattle. The stocking rate used in the model simulation was 2 hectares per animal unit. Annual production ranged from 4.5 to 10.2 t of dry matter per hectare with annual rainfall of 300 to 704 mm, respectively. Total annual intake required per animal unit was estimated at 3.6 ton. Simulated net primary production coincides with reports in the literature, so the model was evaluated successfully.

Ciliary neurotrophic factor activates leptin-like pathways and reduces body fat, without cachexia or rebound weight gain, even in leptin-resistant obesity.

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Lambert, P D; Anderson, K D; Sleeman, M W; Wong, V; Tan, J; Hijarunguru, A; Corcoran, T L; Murray, J D; Thabet, K E; Yancopoulos, G D; Wiegand, S J

2001-04-10

Ciliary Neurotrophic Factor (CNTF) was first characterized as a trophic factor for motor neurons in the ciliary ganglion and spinal cord, leading to its evaluation in humans suffering from motor neuron disease. In these trials, CNTF caused unexpected and substantial weight loss, raising concerns that it might produce cachectic-like effects. Countering this possibility was the suggestion that CNTF was working via a leptin-like mechanism to cause weight loss, based on the findings that CNTF acts via receptors that are not only related to leptin receptors, but also similarly distributed within hypothalamic nuclei involved in feeding. However, although CNTF mimics the ability of leptin to cause fat loss in mice that are obese because of genetic deficiency of leptin (ob/ob mice), CNTF is also effective in diet-induced obesity models that are more representative of human obesity, and which are resistant to leptin. This discordance again raised the possibility that CNTF might be acting via nonleptin pathways, perhaps more analogous to those activated by cachectic cytokines. Arguing strongly against this possibility, we now show that CNTF can activate hypothalamic leptin-like pathways in diet-induced obesity models unresponsive to leptin, that CNTF improves prediabetic parameters in these models, and that CNTF acts very differently than the prototypical cachectic cytokine, IL-1. Further analyses of hypothalamic signaling reveals that CNTF can suppress food intake without triggering hunger signals or associated stress responses that are otherwise associated with food deprivation; thus, unlike forced dieting, cessation of CNTF treatment does not result in binge overeating and immediate rebound weight gain.

Exhaled breath analysis using electronic nose in cystic fibrosis and primary ciliary dyskinesia patients with chronic pulmonary infections

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Joensen, Odin; Paff, Tamara; Haarman, Eric G

2014-01-01

The current diagnostic work-up and monitoring of pulmonary infections may be perceived as invasive, is time consuming and expensive. In this explorative study, we investigated whether or not a non-invasive exhaled breath analysis using an electronic nose would discriminate between cystic fibrosis...... (CF) and primary ciliary dyskinesia (PCD) with or without various well characterized chronic pulmonary infections. We recruited 64 patients with CF and 21 with PCD based on known chronic infection status. 21 healthy volunteers served as controls. An electronic nose was employed to analyze exhaled......, this method significantly discriminates CF patients suffering from a chronic pulmonary P. aeruginosa (PA) infection from CF patients without a chronic pulmonary infection. Further studies are needed for verification and to investigate the role of electronic nose technology in the very early diagnostic workup...

Segmental allotetraploidy and allelic interactions in buffelgrass (Pennisetum ciliare (L.) Link syn. Cenchrus ciliaris L.) as revealed by genome mapping.

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Jessup, R W; Burson, B L; Burow, O; Wang, Y W; Chang, C; Li, Z; Paterson, A H; Hussey, M A

2003-04-01

Linkage analyses increasingly complement cytological and traditional plant breeding techniques by providing valuable information regarding genome organization and transmission genetics of complex polyploid species. This study reports a genome map of buffelgrass (Pennisetum ciliare (L.) Link syn. Cenchrus ciliaris L.). Maternal and paternal maps were constructed with restriction fragment length polymorphisms (RFLPs) segregating in 87 F1 progeny from an intraspecific cross between two heterozygous genotypes. A survey of 862 heterologous cDNAs and gDNAs from across the Poaceae, as well as 443 buffelgrass cDNAs, yielded 100 and 360 polymorphic probes, respectively. The maternal map included 322 RFLPs, 47 linkage groups, and 3464 cM, whereas the paternal map contained 245 RFLPs, 42 linkage groups, and 2757 cM. Approximately 70 to 80% of the buffelgrass genome was covered, and the average marker spacing was 10.8 and 11.3 cM on the respective maps. Preferential pairing was indicated between many linkage groups, which supports cytological reports that buffelgrass is a segmental allotetraploid. More preferential pairing (disomy) was found in the maternal than paternal parent across linkage groups (55 vs. 38%) and loci (48 vs. 15%). Comparison of interval lengths in 15 allelic bridges indicated significantly less meiotic recombination in paternal gametes. Allelic interactions were detected in four regions of the maternal map and were absent in the paternal map.

Neural stem cells in the adult ciliary epithelium express GFAP and are regulated by Wnt signaling

International Nuclear Information System (INIS)

Das, Ani V.; Zhao Xing; James, Jackson; Kim, Min; Cowan, Kenneth H.; Ahmad, Iqbal

2006-01-01

The identification of neural stem cells with retinal potential in the ciliary epithelium (CE) of the adult mammals is of considerable interest because of their potential for replacing or rescuing degenerating retinal neurons in disease or injury. The evaluation of such a potential requires characterization of these cells with regard to their phenotypic properties, potential, and regulatory mechanisms. Here, we demonstrate that rat CE stem cells/progenitors in neurosphere culture display astrocytic nature in terms of expressing glial intermediate neurofilament protein, GFAP. The GFAP-expressing CE stem cells/progenitors form neurospheres in proliferating conditions and generate neurons when shifted to differentiating conditions. These cells express components of the canonical Wnt pathway and its activation promotes their proliferation. Furthermore, we demonstrate that the activation of the canonical Wnt pathway influences neuronal differentiation of CE stem cells/progenitors in a context dependent manner. Our observations suggest that CE stem cells/progenitors share phenotypic properties and regulatory mechanism(s) with neural stem cells elsewhere in the adult CNS

Um caso raro de discinesia ciliar primária associada a heterotaxia A rare case of primary ciliary dyskinesia with heterotaxy

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Cátia Quintela

2009-01-01

Full Text Available A discinesia ciliar primária é uma doença autossómica recessiva caracterizada pela história de infecções de repetição do aparelho respiratório superior e inferior, rinossinusite e bronquite associada a situs inversus completo ou parcial. Os autores apresentam um doente de 78 anos, eurocaucasiano, com rinossinusites, bronquite crónica e dispneia, otite média com défices auditivos, infertilidade, seguido em consulta de gastrenterologia por dispepsia e obstipação há vários anos. Realizou vários exames que mostraram: agenesia frontal direita, espessamento brônquico, bronquiectasias, cego e cólon ascendente localizados na fossa ilíaca esquerda. Excluiu-se imunodeficiência, alergias, fibrose quística e outros. No decurso da investigação concluímos que se tratava de um caso de discinesia ciliar primária. Pela raridade deste caso, decidimos apresentá-lo.Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78-year-old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it.

Gene expression and functional annotation of the human ciliary body epithelia.

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Sarah F Janssen

Full Text Available PURPOSE: The ciliary body (CB of the human eye consists of the non-pigmented (NPE and pigmented (PE neuro-epithelia. We investigated the gene expression of NPE and PE, to shed light on the molecular mechanisms underlying the most important functions of the CB. We also developed molecular signatures for the NPE and PE and studied possible new clues for glaucoma. METHODS: We isolated NPE and PE cells from seven healthy human donor eyes using laser dissection microscopy. Next, we performed RNA isolation, amplification, labeling and hybridization against 44×k Agilent microarrays. For microarray conformations, we used a literature study, RT-PCRs, and immunohistochemical stainings. We analyzed the gene expression data with R and with the knowledge database Ingenuity. RESULTS: The gene expression profiles and functional annotations of the NPE and PE were highly similar. We found that the most important functionalities of the NPE and PE were related to developmental processes, neural nature of the tissue, endocrine and metabolic signaling, and immunological functions. In total 1576 genes differed statistically significantly between NPE and PE. From these genes, at least 3 were cell-specific for the NPE and 143 for the PE. Finally, we observed high expression in the (NPE of 35 genes previously implicated in molecular mechanisms related to glaucoma. CONCLUSION: Our gene expression analysis suggested that the NPE and PE of the CB were quite similar. Nonetheless, cell-type specific differences were found. The molecular machineries of the human NPE and PE are involved in a range of neuro-endocrinological, developmental and immunological functions, and perhaps glaucoma.

Non-invasive measurements of the dynamic changes in the ciliary muscle, crystalline lens morphology, and anterior chamber during accommodation with a high-resolution OCT.

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Esteve-Taboada, José J; Domínguez-Vicent, Alberto; Monsálvez-Romín, Daniel; Del Águila-Carrasco, Antonio J; Montés-Micó, Robert

2017-07-01

The purpose of the study was to assess non-invasively the changes in the anterior chamber eye, crystalline lens morphology, and ciliary muscle during accommodation by means of an anterior chamber optical coherence tomographer (OCT), and correlate them with vergence. Twenty-five eyes of twenty-five healthy subjects, whose mean age was 29.9±7.1 years, were included and measured with an anterior chamber OCT. The central corneal thickness (CCT), anterior chamber depth (ACD), anterior crystalline lens radius of curvature (ALRC), crystalline lens thickness (CLT), and ciliary muscle area (CMA) were measured for each participant at 0, -1, -2, and -3 D of target vergence. A linear model was used to assess the correlation of each eye parameter with the vergence demand. The mean CCT showed no change for all the accommodative stimuli. The mean ACD and ALRC decreased with the vergence, about 4.5 and 30 % at -3 D, respectively. On the contrary, the CLT and CMA showed an opposite tendency, where the mean CLT was increased by 4.0 % and the mean CMA was done by 26% at -3 D. Statistical significant differences (p < 0.001) were obtained among all vergences for each eye metric, except for the CCT (p = 0.76). The ACD and ALRC decreased about 2 and 10 % per dioptre of accommodation, respectively; whereas the CLT and CMA increased about 2 and 9 %, respectively. These results add knowledge regarding the understanding of accommodation and give new perspectives for biomechanics and biometry.

A Low-Cost Method of Ciliary Beat Frequency Measurement Using iPhone and MATLAB: Rabbit Study.

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Chen, Jason J; Lemieux, Bryan T; Wong, Brian J F

2016-08-01

(1) To determine ciliary beat frequency (CBF) using a consumer-grade cellphone camera and MATLAB and (2) to evaluate the effectiveness and accuracy of the proposed method. Prospective animal study. Academic otolaryngology department research laboratory. Five ex vivo tracheal samples were extracted from 3 freshly euthanized (MATLAB programming, the video of the 23°C sample was downsampled to 120, 60, and 30 fps, and Fourier analysis was performed on videos of all frame rates and conditions to determine CBF. CBF of the 23°C sample was also calculated manually frame by frame for verification. Recorded at 240 fps, the CBF at 23°C was 5.03 ± 0.4 Hz, and the CBF at 37°C was 9.08 ± 0.49 Hz (P .05). There is no significant difference between CBF measured via visual inspection and that analyzed by the developed program. Furthermore, all tested acquisition rates are shown to be effective, providing a fast and inexpensive alternative to current CBF measurement protocols. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

Apparent X-linked primary ciliary dyskinesia associated with retinitis pigmentosa and a hearing loss.

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Krawczyński, Maciej R; Dmeńska, Hanna; Witt, Michał

2004-01-01

Three brothers, one 10-year-old and a pair of 14-year-old dizygotic twins--expressed the classical, early-onset retinitis pigmentosa (RP) with typical ophthalmoscopic findings, night blindness, visual field constricted to 10 degrees and flat ERG response. All three brothers were also diagnosed with primary ciliary dyskinesia (PCD) and had recurrent respiratory infections, chronic sinusitis and bronchiectasis. In all of them, resection of the middle lobe of the right lung was performed. A similar clinical picture of coexisting RP and PCD was noted in the brother of the probands' mother. All probands displayed situs solitus. Consistent with the X-linked mode of RP inheritance, there were also three obligatory female carriers of the disorder in this family: the mother of the affected boys, her mother and a daughter of her brother. In all of them, retinitis pigmentosa "sine pigmento" was found with milder but clinically significant symptoms (mild night blindness, visual field constricted to 30 degrees, and scotopic and photopic ERG responses reduced to 30-60%). No extraocular symptoms were detected in any of the heterozygous female carriers. This family presents an example of two rare phenomena: X-linked dominant retinitis pigmentosa (with milder expression in females) and a rare combination of RP with recurrent respiratory infections due to PCD.

Use of a novel cell adhesion method and digital measurement to show stimulus-dependent variation in somatic and oral ciliary beat frequency in Paramecium.

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Bell, Wade E; Hallworth, Richard; Wyatt, Todd A; Sisson, Joseph H

2015-01-01

When Paramecium encounters positive stimuli, the membrane hyperpolarizes and ciliary beat frequency increases. We adapted an established immobilization protocol using a biological adhesive and a novel digital analysis system to quantify beat frequency in immobilized Paramecium. Cells showed low mortality and demonstrated beat frequencies consistent with previous studies. Chemoattractant molecules, reduction in external potassium, and posterior stimulation all increased somatic beat frequency. In all cases, the oral groove cilia maintained a higher beat frequency than mid-body cilia, but only oral cilia from cells stimulated with chemoattactants showed an increase from basal levels. © 2014 The Author(s) Journal of Eukaryotic Microbiology © 2014 International Society of Protistologists.

Expression and prognostic value of putative cancer stem cell markers CD117 and CD15 in choroidal and ciliary body melanoma.

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Lukenda, Adrian; Dotlic, Snjezana; Vukojevic, Nenad; Saric, Borna; Vranic, Semir; Zarkovic, Kamelija

2016-03-01

The aim of the present study was to immunohistochemically investigate the expression and prognostic significance of putative cancer stem cell markers CD117 (c-kit), CD34, CD20 and CD15 in a cohort of patients with primary choroidal and ciliary body melanoma. The immunohistochemical expression of these markers was evaluated using 3,3'-diaminobenzidine tetrahydrochloride (DAB) and 3-amino-9-ethylcarbazole (AEC) chromogens on paraffin-embedded tissue samples from 40 patients who underwent enucleation in the period from 1985 through 2000. Thirty-one patients had adequate tissue specimens for the analysis. CD117 overexpression was observed in 12 of the 31 samples (39%) when AEC chromogen was used and in 14 of 26 (54%) samples when DAB was used. CD15 positivity was seen in three out of 30 (10%) samples with AEC and in six out of 26 (23%) samples with DAB. CD20 and CD34 exhibited no positivity in the tested samples. During the average follow-up time of 8.7 years (range 0.5-22 years), 17 patients (55%) died due to metastatic disease. The Kaplan-Meier plots showed a significantly shorter overall and disease-free survival in CD117-positive patients when the AEC chromogen was used. CD15 expression was not associated with patients' survival. In multivariate analysis, patients expressing the CD117 AEC had 4.13 times higher risk of lethal outcome in comparison with CD117 AEC negative patients. Our retrospective cohort study has for the first time demonstrated a small proportion of CD15-positive uveal melanomas. CD117 AEC overexpression was associated with a worse outcome in patients with choroidal and ciliary body melanoma. Further studies should confirm the validity of these observations and their potential for targeted treatment modalities. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/

Central retinal and posterior ciliary artery occlusion after particle embolization of the external carotid artery system.

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Mames, R N; Snady-McCoy, L; Guy, J

1991-04-01

A 15-year-old boy underwent neuroradiologic embolization of the left internal maxillary artery with polyvinyl alcohol to stop traumatic epistaxis after failure of surgical clipping and nasal packing. Selective catheterization of the external carotid artery before embolization showed a faint choroidal blush. Although the procedure provided hemostasis, embolization to the central retinal artery and ciliary arteries resulted in loss of vision. The route of the emboli to the eye was via the anastomotic network of the lacrimal artery supplied by the external carotid artery system. Neuroradiologic embolization of the external carotid artery is an effective mode of therapy for dural-cavernous fistulas when fed by the external carotid artery system. Because the blood flow to the brain and eye is predominantly supplied by the internal carotid artery, embolization of the external carotid artery is considered relatively safe. The authors document the importance of recognition of the choroidal blush during selective external carotid artery angiography as a sign of collateral blood flow to the eye. Physicians and patients need to be aware of the risk of blindness as a complication of external carotid artery embolization when this sign is present.

The effects of the stem cell on ciliary regeneration of injured rabbit sinonasal epithelium.

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Kavuzlu, Ali; Tatar, Emel Çadallı; Karagöz, Tuğba; Pınarlı, Ferda Alpaslan; Tatar, İlkan; Bayır, Ömer; Korkmaz, Mehmet Hakan

2017-08-01

Defects in mucosal healing after sinonasal surgery cause infection, scar formation causing obstruction, relapse of the disease within a shorter period and revision surgery. The present study aimed to create a functional ciliated epithelium using a stem cell and stem cell sheet of adipose tissue origin and to show such regeneration ultra-structurally on experimentally injured rabbit nasal epithelium. This was an experimental animal study and basic research. A total of 18 white New Zealand rabbits were divided into three groups. The medial wall of the maxillary sinus of the subjects was peeled off bilaterally. No additional procedure was applied to the subjects in Group 1. In Group 2, adipose tissue-derived mesenchymal stem cell was implanted on the wound edges of the subjects. In Group 3, a stem cell sheet of three layers was laid onto the defect area. All subjects were killed after 3 weeks. The presence of the stem cell stained with bromo-deoxyuridine was assessed with a light microscope, whereas cilia density, ciliated orientation and cilia structure were evaluated with a scanning electron microscope. Ciliary densities in Group 2 and Group 3 were statistically superior compared to the control group (p stem cell increased the healing of the injured maxillary sinus mucosa of the rabbits in terms of cilia presence, density and morphology regardless of the implementation technique. Level of evidence NA.

Hand-held tidal breathing nasal nitric oxide measurement--a promising targeted case-finding tool for the diagnosis of primary ciliary dyskinesia

DEFF Research Database (Denmark)

Marthin, June Kehlet; Nielsen, Kim Gjerum

2013-01-01

BACKGROUND: Nasal nitric oxide (nNO) measurement is an established first line test in the work-up for primary ciliary dyskinesia (PCD). Tidal breathing nNO (TB-nNO) measurements require minimal cooperation and are potentially useful even in young children. Hand-held NO devices are becoming...... increasingly widespread for asthma management. Therefore, we chose to assess whether hand-held TB-nNO measurements reliably discriminate between PCD, and Healthy Subjects (HS) and included Cystic Fibrosis (CF) patients as a disease control group known to have intermediate nNO levels. METHODS: In this cross...... sectional, single centre, single occasion, proof-of-concept study in children and adults with PCD and CF, and in HS we compared feasibility, success rates, discriminatory capacity, repeatability and agreement between a hand-held electrochemical device equipped with a nNO software application sampling...

Phylogenetic analysis of the Neks reveals early diversification of ciliary-cell cycle kinases.

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Jeremy D K Parker

2007-10-01

Full Text Available NIMA-related kinases (Neks have been studied in diverse eukaryotes, including the fungus Aspergillus and the ciliate Tetrahymena. In the former, a single Nek plays an essential role in cell cycle regulation; in the latter, which has more than 30 Neks in its genome, multiple Neks regulate ciliary length. Mammalian genomes encode an intermediate number of Neks, several of which are reported to play roles in cell cycle regulation and/or localize to centrosomes. Previously, we reported that organisms with cilia typically have more Neks than organisms without cilia, but were unable to establish the evolutionary history of the gene family.We have performed a large-scale analysis of the Nek family using Bayesian techniques, including tests of alternate topologies. We find that the Nek family had already expanded in the last common ancestor of eukaryotes, a ciliated cell which likely expressed at least five Neks. We suggest that Neks played an important role in the common ancestor in regulating cilia, centrioles, and centrosomes with respect to mitotic entry, and that this role continues today in organisms with cilia. Organisms that lack cilia generally show a reduction in the number of Nek clades represented, sometimes associated with lineage specific expansion of a single clade, as has occurred in the plants.This is the first rigorous phylogenetic analysis of a kinase family across a broad array of phyla. Our findings provide a coherent framework for the study of Neks and their roles in coordinating cilia and cell cycle progression.

Studies on SO/sub 2/, NO/sub 2/ and NH/sub 3/: effect on ciliary activity in rabbit trachea of single in vitro exposure and resorption in rabbit nasal cavity

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Dalhamm, T; Sjoeholm, J

1963-01-01

Absorption in nasal cavity was tested by drawing gas through tracheal cannula at 1 liter/min for 45 min (...simple and is not intended to give accurate quantitative results.). Both SO/sub 2/ and NH/sub 3/ were strongly absorbed, even at high concentrations, whereas NO/sub 2/ was not. Required concentration to arrest ciliary activity after 5 min was 500 to 1000 mg/liter for NH/sub 3/, 150 to 200 for NO/sub 2/, and 20 to 30 for SO/sub 2/. Some evidence of dose-time-effect for this action was observed.

Clinical outcomes of Ahmed glaucoma valve in anterior chamber versus ciliary sulcus.

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Bayer, A; Önol, M

2017-04-01

PurposeTo evaluate the outcomes of Ahmed glaucoma valve (AGV) tube insertion through the anterior chamber angle (ACA) or through the ciliary sulcus (CS).Patients and methodsIn this case-control study, we retrospectively reviewed the charts of consecutive glaucoma patients who had undergone AGV implantation either through the ACA or the CS between March 2009 and December 2014. The main outcome measures were intraocular pressure (IOP), number of glaucoma medications prescribed, best corrected visual acuity (BCVA), glaucoma type, success rate, complications, and survival ratios. Statistical analysis was carried out using SPSS.ResultsThere were 68 eyes in the ACA group and 35 eyes in the CS group. There were no significant differences between the groups for age, sex, laterality, IOP, preoperative glaucoma medication number, BCVA or glaucoma type (P>0.05). The postoperative follow-up period was 27.2±16.5 months and 30.2±17.7 months for the ACA and the CS groups (P=0.28); IOP values were significantly reduced at the last visit to 16.4±7.2 mm Hg and 14.4±6.8 mm Hg. The difference in the last-visit IOP between the groups was not significant (P=0.06), but the IOP reduction ratio was higher in the CS group (P=0.03). There was no significant difference in the number of postoperative medications (P=0.18). Postoperative complications were similar, but the incidence of flat anterior chamber was higher in the ACA group (P=0.05).ConclusionsThe use of an AGV can control IOP in the majority of cases whether placed in the ACA or the CS. The IOP reduction ratio seemed to be higher in the CS group.

Evaluation of pulmonary disease using static lung volumes in primary ciliary dyskinesia.

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Pifferi, Massimo; Bush, Andrew; Pioggia, Giovanni; Caramella, Davide; Tartarisco, Gennaro; Di Cicco, Maria; Zangani, Marta; Chinellato, Iolanda; Maggi, Fabrizio; Tezza, Giovanna; Macchia, Pierantonio; Boner, Attilio

2012-11-01

In primary ciliary dyskinesia (PCD) lung damage is usually evaluated by high-resolution CT (HRCT). To evaluate whether HRCT abnormalities and Pseudomonas aeruginosa infection were better predicted by spirometry or plethysmography. A cross-sectional study performed in consecutive patients with PCD who underwent sputum culture, spirometry, plethysmography and HRCT within 48 h. Principal component analysis and soft computing were used for data evaluation. Fifty patients (26 children) were studied. P aeruginosa infection was found in 40% of the patients and bronchiectasis in 88%. There was a correlation between infection with P aeruginosa and extent of bronchiectasis (p=0.009; r =0.367) and air-trapping (p=0.03; r =0.315). Moreover, there was an association between infection with P aeruginosa and residual volume (RV) values >150% (p=0.04) and RV/total lung capacity (TLC) ratio >140% (p=0.001), but not between infection with P aeruginosa and forced expiratory volume in 1 s (FEV(1))<80%, or forced expiratory flow between 25% and 75% of forced vital capacity (FVC) (FEF(25-75%))<70% or FEV(1)/FVC<70% (<80% in children). Severity of the total lung impairment on chest HRCT directly correlated with RV when expressed as per cent predicted (p=0.003; r =0.423), and RV/TLC (p<0.001; r =0.513) or when expressed as z scores (p=0.002, r =0.451 and p<0.001, r =0.536 respectively). Principal component analysis on plethysmographic but not on spirometry data allowed recognition of different severities of focal air trapping, atelectasis and extent of bronchiectasis. Plethysmography better predicts HRCT abnormalities than spirometry. Whether it might be a useful test to define populations of patients with PCD who should or should not have HRCT scans requires further longitudinal studies.

Protective effect of ciliary neurotrophic factor (CNTF) in a model of endotoxic shock: action mechanisms and role of CNTF receptor alpha.

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Demitri, M T; Benigni, F; Meazza, C; Zinetti, M; Fratelli, M; Villa, P; Acheson, A; Panayotatos, N; Ghezzi, P

1998-01-01

Ciliary neurotrophic factor (CNTF) inhibits the production of tumor necrosis factor (TNF) in lipopolysaccharide (LPS)-treated mice and protects against LPS lethality when coadministered with its soluble receptor (sCNTFR alpha). Both of these activities are abolished in adrenalectomized (ADX) mice. LPS-induced pulmonary polymorphonuclear neutrophil (PMN) infiltration and nitric oxide (NO) production were also inhibited by CNTF + sCNTFR alpha but not by CNTF alone. sCNTFR alpha did not alter the clearance or tissue distribution of CNTF. Furthermore, CNTF variants coadministered with sCNTFR alpha protected against LPS toxicity in a manner related to their affinity for the beta components of CNTFR. Thus, inhibition of TNF production and protection against LPS lethality by CNTF/sCNTFR alpha require an intact hypothalamus-pituitary-adrenal axis (HPAA) and may be mediated by endogenous glucocorticoids. This protective effect is, at least in part, due to the inhibition of PMN infiltration and NO production, and appears to be mediated by cells displaying only beta-receptor subtypes.

The ciliary marginal zone of the zebrafish retina: clonal and time-lapse analysis of a continuously growing tissue.

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Wan, Yinan; Almeida, Alexandra D; Rulands, Steffen; Chalour, Naima; Muresan, Leila; Wu, Yunmin; Simons, Benjamin D; He, Jie; Harris, William A

2016-04-01

Clonal analysis is helping us understand the dynamics of cell replacement in homeostatic adult tissues (Simons and Clevers, 2011). Such an analysis, however, has not yet been achieved for continuously growing adult tissues, but is essential if we wish to understand the architecture of adult organs. The retinas of lower vertebrates grow throughout life from retinal stem cells (RSCs) and retinal progenitor cells (RPCs) at the rim of the retina, called the ciliary marginal zone (CMZ). Here, we show that RSCs reside in a niche at the extreme periphery of the CMZ and divide asymmetrically along a radial (peripheral to central) axis, leaving one daughter in the peripheral RSC niche and the other more central where it becomes an RPC. We also show that RPCs of the CMZ have clonal sizes and compositions that are statistically similar to progenitor cells of the embryonic retina and fit the same stochastic model of proliferation. These results link embryonic and postembryonic cell behaviour, and help to explain the constancy of tissue architecture that has been generated over a lifetime. © 2016. Published by The Company of Biologists Ltd.

A relação entre o comprimento dos processos ciliares medidos pela biomicroscopia ultra-sônica e a duração, localização e gravidade das uveítes The relationship between the length of ciliary processes as measured by ultrasound biomicroscopy and the duration, localization and severity of uveitis

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Daniella Socci da Costa

2006-06-01

Full Text Available OBJETIVO: Determinar a relação entre o comprimento dos processos ciliares com a duração, localização e gravidade das uveítes. MÉTODOS: Foram analisados em estudo prospectivo, 58 indivíduos, incluindo pacientes com diferentes estágios de uveíte e indivíduos sem a doença (112 olhos, sendo 18 normais, no período de agosto de 2001 a agosto de 2002, no Departamento de Uveítes do Cole Eye Institute da Cleveland Clinic Foundation - Ohio - EUA. Todos os pacientes foram referidos para o exame de biomicroscopia ultra-sônica, pelo Departamento de Uveítes após exame oftalmológico de rotina. O aparelho modelo 840 (Zeiss-Humphrey com transdutor de 50 MHz foi utilizado para análise dos processos ciliares sob anestesia tópica e técnica de imersão. RESULTADOS: Em relação à etiologia das uveítes, a de maior freqüência foi a idiopática (27,8%. As uveítes recorrentes, agressivas e difusas levaram ao significante dano nos processos ciliares. A maior perda significativa na medida dos processos ciliares foi encontrada no quadrante inferior e as maiores medidas foram encontradas em olhos normais e no quadrante temporal. CONCLUSÃO: A biomicroscopia ultra-sônica mostrou ser método útil para avaliar as alterações anatômicas encontradas nos processos ciliares dos pacientes com uveíte. De acordo com estes achados, podemos criar recomendações para futuros trabalhos, que nos ajudem a avaliar a necessidade destes pacientes vir a receber tratamento mais agressivo em qualquer sinal de inflamação, com o objetivo de prevenir futuro dano e eventual hipotonia.PURPOSE: To determine the relationship between the length of ciliary processes as measured by ultrasound biomicroscopy and the duration, localization and severity of uveitis. METHODS: We analyzed in this prospective study, 58 individuals including patients with different stages of uveitis and normal patients (112 eyes including 18 normal, between August 2001 and August 2002, at the

Long-term Results of the UCSF-LBNL Randomized Trial: Charged Particle With Helium Ion Versus Iodine-125 Plaque Therapy for Choroidal and Ciliary Body Melanoma

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Mishra, Kavita K., E-mail: Kavita.mishra@ucsf.edu [Department of Radiation Oncology, University of California-San Francisco, San Francisco, California (United States); Quivey, Jeanne M.; Daftari, Inder K. [Department of Radiation Oncology, University of California-San Francisco, San Francisco, California (United States); Lawrence Berkeley National Laboratory, Berkeley, California (United States); Weinberg, Vivian [Department of Radiation Oncology, University of California-San Francisco, San Francisco, California (United States); Cole, Tia B. [The Tumori Foundation, San Francisco, California (United States); Patel, Kishan [Department of Radiation Oncology, University of California-San Francisco, San Francisco, California (United States); Castro, Joseph R.; Phillips, Theodore L. [Department of Radiation Oncology, University of California-San Francisco, San Francisco, California (United States); Lawrence Berkeley National Laboratory, Berkeley, California (United States); Char, Devron H. [The Tumori Foundation, San Francisco, California (United States); Department of Ophthalmology, University of California-San Francisco, San Francisco, California (United States); Department of Ophthalmology, Stanford University, Palo Alto, California (United States)

2015-06-01

Purpose: Relevant clinical data are needed given the increasing national interest in charged particle radiation therapy (CPT) programs. Here we report long-term outcomes from the only randomized, stratified trial comparing CPT with iodine-125 plaque therapy for choroidal and ciliary body melanoma. Methods and Materials: From 1985 to 1991, 184 patients met eligibility criteria and were randomized to receive particle (86 patients) or plaque therapy (98 patients). Patients were stratified by tumor diameter, thickness, distance to disc/fovea, anterior extension, and visual acuity. Tumors close to the optic disc were included. Local tumor control, as well as eye preservation, metastases due to melanoma, and survival were evaluated. Results: Median follow-up times for particle and plaque arm patients were 14.6 years and 12.3 years, respectively (P=.22), and for those alive at last follow-up, 18.5 and 16.5 years, respectively (P=.81). Local control (LC) for particle versus plaque treatment was 100% versus 84% at 5 years, and 98% versus 79% at 12 years, respectively (log rank: P=.0006). If patients with tumors close to the disc (<2 mm) were excluded, CPT still resulted in significantly improved LC: 100% versus 90% at 5 years and 98% versus 86% at 12 years, respectively (log rank: P=.048). Enucleation rate was lower after CPT: 11% versus 22% at 5 years and 17% versus 37% at 12 years, respectively (log rank: P=.01). Using Cox regression model, likelihood ratio test, treatment was the most important predictor of LC (P=.0002) and eye preservation (P=.01). CPT was a significant predictor of prolonged disease-free survival (log rank: P=.001). Conclusions: Particle therapy resulted in significantly improved local control, eye preservation, and disease-free survival as confirmed by long-term outcomes from the only randomized study available to date comparing radiation modalities in choroidal and ciliary body melanoma.

Control of ventricular ciliary beating by the Melanin Concentrating Hormone-expressing neurons of the lateral hypothalamus : a functional imaging survey.

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Gregory eConductier

2013-11-01

Full Text Available The cyclic peptide Melanin-Concentrating Hormone (MCH is known to control a large number of brain functions in mammals such as food intake and metabolism, stress response, anxiety, sleep/wake cycle, memory and reward. Based on neuroanatomical and electrophysiological studies these functions were attributed to neuronal circuits expressing MCHR1, the single MCH receptor in rodents. In complement to our recently published work (Conductier et al. 2013 we provided here new data regarding the action of MCH on ependymocytes in the mouse brain. First, we establish that MCHR1 mRNA is expressed in the ependymal cells of the third ventricle epithelium. Second, we demonstated a tonic control of MCH-expressing neurons on ependymal cilia beat frequency using in vitro optogenics. Finally, we performed in vivo measurements of CSF flow using fluorescent micro-beads in wild-type and MCHR1 knockout mice. Collectively, our results demonstrated that MCH-expressing neurons modulate ciliary beating of ependymal cells at the third ventricle and could contribute to maintain cerebro-spinal fluid homeostasis.

Does the disruption of horizontal anterior ciliary vessels affect the blood-aqueous barrier function?

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Pan, Meihua; Yang, Mei; Xie, Renyi; Zhao, Zhimin; Huang, Xingxing

2017-12-01

To investigate the significance of the anterior ciliary vessels (ACVs) preservation during the conventional horizontal strabismus surgery. Patients (≥ 8 years) with horizontal strabismus were randomly allocated into group 1 (with ACV preservation) and group 2 (without ACV preservation). The surgical eyes in group 1 were further divided into group A (one rectus muscle operated) and group B (two rectus muscles operated). Similarly, eyes in group 2 were divided into group C (one rectus muscle operated) and group D (two rectus muscles operated). The success rate of ACV preservation was calculated. The anterior chamber flare measurements of each eye by laser flare photometry were recorded on the day prior to and after operation. The flare values between groups and between pre- and post-operation in each group were compared by one-way analysis of variance and a paired t-test respectively. In groups A and B, the success rate of ACV preservation was 82% (27/33) and 70% (28/40)respectively, and the flare values between pre- and post-operation showed no significant differences(4.378 ± 1.527, 4.544 ± 1.452, P = 0.526; 4.625 ± 1.090, 4.989 ± 1.468, P = 0.101 respectively). However, the postoperative values were significantly increased in group C and group D(4.661 ± 1.031, 5.039 ± 1.310, P = 0.025; 4.933 ± 1.691, 5.502 ± 1.430, P = 0.000 respectively). The postoperative flare readings of group D were significantly higher than group B, while group A and group C had no significant variation. ACV preservation probably has clinical significance in reducing the undesirable influence on the blood-aqueous barrier.

Nueva variante de abordaje al surco ciliar en la fijación transescleral de lentes de cámara posterior New variant of approach to the ciliary sulcus in the transcleral fixation of posterior chamber lens

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Ileana Vila Dópico

2002-06-01

Full Text Available Se expone una variante de abordaje al surco ciliar para la fijación del lente intraocular de cámara posterior, que está a nuestro alcance, en pacientes en que no existe soporte capsular o este es insuficiente. Se describe la técnica. Se realizó en 17 pacientes de los que se obtuvo mejoría visual en todos los casos (20/50-20/20 con un seguimiento promedio de 12 meses. Esta variante tiene la ventaja de evitar el paso de la aguja por el surco ciliar a ciegas, pues se utiliza el abordaje a través de la esclera y con ello evitamos dañar estructuras oculares aledañas evitando el riesgo de complicaciones por lo que se logra la recuperación anatómica y funcional en todos los casos.A variant to approach the ciliary sulcus for fixating the posterior chamber intraocular lens, which is within our reach, in patients with insufficient or no capsular support, is explained. The technique is described here and it was applied to 17 patients. Visual improvement was obtained in all cases (20/50-20/20 with an average follow-up of 12 months. This variant allows to prevent the blind passage of the needle through the ciliary sulcus, since it is approached via the sclera, avoiding to damage adjoining ocular structures and to have complications, and making possible the anatomic and functional recovery of all patients.

An Automated Measurement of Ciliary Beating Frequency using a Combined Optical Flow and Peak Detection.

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Kim, Woojae; Han, Tae Hwa; Kim, Hyun Jun; Park, Man Young; Kim, Ku Sang; Park, Rae Woong

2011-06-01

The mucociliary transport system is a major defense mechanism of the respiratory tract. The performance of mucous transportation in the nasal cavity can be represented by a ciliary beating frequency (CBF). This study proposes a novel method to measure CBF by using optical flow. To obtain objective estimates of CBF from video images, an automated computer-based image processing technique is developed. This study proposes a new method based on optical flow for image processing and peak detection for signal processing. We compare the measuring accuracy of the method in various combinations of image processing (optical flow versus difference image) and signal processing (fast Fourier transform [FFT] vs. peak detection [PD]). The digital high-speed video method with a manual count of CBF in slow motion video play, is the gold-standard in CBF measurement. We obtained a total of fifty recorded ciliated sinonasal epithelium images to measure CBF from the Department of Otolaryngology. The ciliated sinonasal epithelium images were recorded at 50-100 frames per second using a charge coupled device camera with an inverted microscope at a magnification of ×1,000. The mean square errors and variance for each method were 1.24, 0.84 Hz; 11.8, 2.63 Hz; 3.22, 1.46 Hz; and 3.82, 1.53 Hz for optical flow (OF) + PD, OF + FFT, difference image [DI] + PD, and DI + FFT, respectively. Of the four methods, PD using optical flow showed the best performance for measuring the CBF of nasal mucosa. The proposed method was able to measure CBF more objectively and efficiently than what is currently possible.

Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD).

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Behan, Laura; Leigh, Margaret W; Dell, Sharon D; Dunn Galvin, Audrey; Quittner, Alexandra L; Lucas, Jane S

2017-09-01

Quality of life (QOL)-primary ciliary dyskinesia (PCD) is the first disease-specific, health-related QOL instrument for PCD. Psychometric validation of QOL-PCD assesses the performance of this measure in adults, including its reliability, validity and responsiveness to change. Seventy-two adults (mean (range) age: 33 years (18-79 years); mean (range) FEV 1 % predicted: 68 (26-115)) with PCD completed the 49-item QOL-PCD and generic QOL measures: Short-Form 36 Health Survey, Sino-Nasal Outcome Test 20 (SNOT-20) and St George Respiratory Questionnaire (SGRQ)-C. Thirty-five participants repeated QOL-PCD 10-14 days later to measure stability or reproducibility of the measure. Multitrait analysis was used to evaluate how the items loaded on 10 hypothesised scales: physical, emotional, role and social functioning, treatment burden, vitality, health perceptions, upper respiratory symptoms, lower respiratory symptoms and ears and hearing symptoms. This analysis of item-to-total correlations led to 9 items being dropped; the validated measure now comprises 40 items. Each scale had excellent internal consistency (Cronbach's α: 0.74 to 0.94). Two-week test-retest demonstrated stability for all scales (intraclass coefficients 0.73 to 0.96). Significant correlations were obtained between QOL-PCD scores and age and FEV 1 . Strong relationships were also found between QOL-PCD scales and similar constructs on generic questionnaires, for example, lower respiratory symptoms and SGRQ-C (r=0.72, pmeasures of different constructs. QOL-PCD has demonstrated good internal consistency, test-retest reliability, convergent and divergent validity. QOL-PCD offers a promising tool for evaluating new therapies and for measuring symptoms, functioning and QOL during routine care. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Discinesia ciliar primária: quando o pediatra deve suspeitar e como diagnosticar? Primary ciliary dyskinesia: when the pediatrician must suspect and how to do the diagnosis?

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Mary Anne K. Olm

2007-12-01

Full Text Available OBJETIVO: Revisar a discinesia ciliar primária (DCP quanto aos seus aspectos ultra-estruturais, discriminar os defeitos ciliares primários dos secundários, descrever o quadro clínico, os testes laboratoriais de triagem e de diagnóstico disponíveis, bem como seu manejo clínico. FONTE DE DADOS: Pesquisa nas bases de dados Medline, Lilacs e SciELO, no período de 1980 a 2007. SÍNTESE DOS DADOS: A DCP é uma doença autossômica recessiva que compromete a estrutura e/ou a função ciliar e, conseqüentemente, o transporte mucociliar. As manifestações clínicas envolvem o trato respiratório superior e inferior, com infecções recorrentes do ouvido médio, seios paranasais e pulmonares, que podem evoluir para bronquiectasias. Outras manifestações incluem situs inversus totalis e infertilidade masculina. O diagnóstico deve ser suspeitado pelos pediatras em várias situações: recém-nascidos de termo com desconforto respiratório sem causa aparente; neonatos portadores de dextrocardia; lactentes com tosse persistente e/ou infecções otorrinolaringológicas de repetição, excluindo-se as imunodeficiências e a fibrose cística; crianças com asma atípica e as com bronquiectasias sem causa definida. Os testes de triagem diagnóstica são os da sacarina e do óxido nítrico nasal. As avaliações do defeito ultra-estrutural e funcional exigem análise por microscopia eletrônica e da freqüência e formato da onda de batimento ciliar. CONCLUSÕES: A DCP, apesar da baixa prevalência, é pouco diagnosticada pelas dificuldades de estabelecer o diagnóstico definitivo do defeito ciliar devido à complexidade da investigação laboratorial e pela falta de reconhecimento da doença pelos médicos. A suspeita clínica e o diagnóstico precoce são fundamentais para reduzir a morbidade e prevenir o desenvolvimento de complicações.OBJECTIVE: To review primary ciliary dyskinesia (PCD and its ultrastructural aspects, to differentiate primary

Intraocular (Eye) Melanoma—Health Professional Version

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Intraocular (uveal) melanoma of the uveal tract (iris, ciliary body, and choroid), though rare, is the most common primary intraocular malignancy in adults. Find evidence-based information on intraocular melanoma treatment.

Cistos primários do epitélio pigmentar da íris e corpo ciliar: aspectos de biomicroscopia ultra-sônica Primary cysts of the iris and ciliary body pigment epithelium: ultrasound biomicroscopy features

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Bernadete Ayres

2000-10-01

Full Text Available Objetivo: Descrever as características, incidência e distribuição dos cistos primários de epitélio pigmentar de íris e corpo ciliar ao exame de biomicroscopia ultra-sônica, que devem ser diferenciados de lesões sólidas. Métodos: Foram estudados de modo retrospectivo os prontuários de 73 pacientes, 82 olhos, com diagnóstico ecográfico de cisto primário de íris ou corpo ciliar durante o período de janeiro/97 a dezembro/99. Utilizou-se o biomicroscópio ultra-sônico, aplicando técnicas padronizadas de imersão. Resultados: À biomicroscopia ultra-sônica os cistos caracterizaram-se por apresentarem paredes finas e regulares, e conteúdo anecóico. Quarenta e oito pacientes (65,7% eram do sexo feminino. A maior incidência (28,8% ocorreu para o grupo incluído no intervalo de 20 a 29 anos de idade. Observou-se uma característica distribuição, predominantemente nos quadrantes temporais inferiores. Conclusões: A biomicroscopia ultra-sônica mostrou-se útil no diagnóstico de cistos primários do epitélio pigmentar da íris e do corpo ciliar, auxiliando na diferenciação de patologias tumorais e avaliando possíveis complicações. O conhecimento dos critérios ecográficos e da distribuição epidemiológica facilitam o diagnóstico destas lesões.Purpose: To describe the ultrasound biomicroscopic characteristics, incidence, distribution and location of primary cysts of the iris and ciliary body pigment epithelium, and to differentiate them from solid lesions. Methods: A retrospective study was performed through a review of charts of 73 patients, 82 eyes, with echographic diagnosis of primary cysts of the iris and ciliary body pigment epithelium during a 36-month period (January/97 through December/99. All examinations were performed using an ultrasound biomicroscope applying standard immersion techniques. Results: Ultrasound biomicroscopy revealed typical findings of the primary cysts of the pigment epithelium such as thin

Super-resolution imaging of ciliary microdomains in isolated olfactory sensory neurons using a custom two-color stimulated emission depletion microscope

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Meyer, Stephanie A.; Ozbay, Baris N.; Potcoava, Mariana; Salcedo, Ernesto; Restrepo, Diego; Gibson, Emily A.

2016-06-01

We performed stimulated emission depletion (STED) imaging of isolated olfactory sensory neurons (OSNs) using a custom-built microscope. The STED microscope uses a single pulsed laser to excite two separate fluorophores, Atto 590 and Atto 647N. A gated timing circuit combined with temporal interleaving of the different color excitation/STED laser pulses filters the two channel detection and greatly minimizes crosstalk. We quantified the instrument resolution to be ˜81 and ˜44 nm, for the Atto 590 and Atto 647N channels. The spatial separation between the two channels was measured to be under 10 nm, well below the resolution limit. The custom-STED microscope is incorporated onto a commercial research microscope allowing brightfield, differential interference contrast, and epifluorescence imaging on the same field of view. We performed immunolabeling of OSNs in mice to image localization of ciliary membrane proteins involved in olfactory transduction. We imaged Ca2+-permeable cyclic nucleotide gated (CNG) channel (Atto 594) and adenylyl cyclase type III (ACIII) (Atto 647N) in distinct cilia. STED imaging resolved well-separated subdiffraction limited clusters for each protein. We quantified the size of each cluster to have a mean value of 88±48 nm and 124±43 nm, for CNG and ACIII, respectively. STED imaging showed separated clusters that were not resolvable in confocal images.

Whole-Exome Sequencing Identified a Novel Compound Heterozygous Mutation of LRRC6 in a Chinese Primary Ciliary Dyskinesia Patient

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Lv Liu

2018-01-01

Full Text Available Primary ciliary dyskinesia (PCD is a clinical rare peculiar disorder, mainly featured by respiratory infection, tympanitis, nasosinusitis, and male infertility. Previous study demonstrated it is an autosomal recessive disease and by 2017 almost 40 pathologic genes have been identified. Among them are the leucine-rich repeat- (LRR- containing 6 (LRRC6 codes for a 463-amino-acid cytoplasmic protein, expressed distinctively in motile cilia cells, including the testis cells and the respiratory epithelial cells. In this study, we applied whole-exome sequencing combined with PCD-known genes filtering to explore the genetic lesion of a PCD patient. A novel compound heterozygous mutation in LRRC6 (c.183T>G/p.N61K; c.179-1G>A was identified and coseparated in this family. The missense mutation (c.183T>G/p.N61K may lead to a substitution of asparagine by lysine at position 61 in exon 3 of LRRC6. The splice site mutation (c.179-1G>A may cause a premature stop codon in exon 4 and decrease the mRNA levels of LRRC6. Both mutations were not present in our 200 local controls, dbSNP, and 1000 genomes. Three bioinformatics programs also predicted that both mutations are deleterious. Our study not only further supported the importance of LRRC6 in PCD, but also expanded the spectrum of LRRC6 mutations and will contribute to the genetic diagnosis and counseling of PCD patients.

Effects of glaucoma medications and preservatives on cultured human trabecular meshwork and non-pigmented ciliary epithelial cell lines.

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Ammar, David A; Kahook, Malik Y

2011-10-01

We investigated the potential cytotoxicity of various topical ophthalmic glaucoma formulations containing different preservatives in cultured human trabecular meshwork (TM) and non-pigmented ciliary epithelial (NPCE) cell lines. We tested 0.004% travoprost preserved with either 0.015% benzalkonium chloride (BAK), sofZia or 0.001% Polyquad (PQ); and 0.005% latanoprost preserved with 0.020% BAK. We also tested a range of BAK concentrations in balanced salt solution (BSS). TM cells were treated for 10 min at 37°C with solutions diluted 1:10 to mimic the reduced penetration of topical preparations to the anterior chamber. Viability was determined by the uptake of the fluorescent vital dye calcein-AM (n = 6). BAK solutions (diluted 1:10) demonstrated a dose-dependent reduction in cell viability in both cell types (TM and NPCE). With a 1:10 dilution of 0.020% BAK, there were significantly more living NPCE cells (89 ± 6%) than TM cells (57 ± 6%; p < 0.001). In TM cells, travoprost + BAK had statistically fewer live cells (83 ± 5%) than both travoprost + sofZia (97 ± 5%) and travoprost + PQ (97 ± 6%; p < 0.05). Compared with BSS-treated NPCE cells, travoprost had statistically fewer live cells (p < 0.05) when preserved with BAK (85 ± 16%), sofZia (91 ± 6%) or PQ (94 ± 2%). These results demonstrate that substitution of BAK from topical ophthalmic drugs results in greater viability of cultured TM cells, the cells involved in the conventional outflow pathway. Cultured NPCE, responsible for aqueous inflow, appear more resilient to BAK.

In vitro assessment of TAT — Ciliary Neurotrophic Factor therapeutic potential for peripheral nerve regeneration

International Nuclear Information System (INIS)

Barbon, Silvia; Stocco, Elena; Negro, Alessandro; Dalzoppo, Daniele; Borgio, Luca; Rajendran, Senthilkumar; Grandi, Francesca; Porzionato, Andrea; Macchi, Veronica; De Caro, Raffaele

2016-01-01

In regenerative neurobiology, Ciliary Neurotrophic Factor (CNTF) is raising high interest as a multifunctional neurocytokine, playing a key role in the regeneration of injured peripheral nerves. Despite its promising trophic and regulatory activity, its clinical application is limited by the onset of severe side effects, due to the lack of efficient intracellular trafficking after administration. In this study, recombinant CNTF linked to the transactivator transduction domain (TAT) was investigated in vitro and found to be an optimized fusion protein which preserves neurotrophic activity, besides enhancing cellular uptake for therapeutic advantage. Moreover, a compelling protein delivery method was defined, in the future perspective of improving nerve regeneration strategies. Following determination of TAT-CNTF molecular weight and concentration, its specific effect on neural SH-SY5Y and PC12 cultures was assessed. Cell proliferation assay demonstrated that the fusion protein triggers PC12 cell growth within 6 h of stimulation. At the same time, the activation of signal transduction pathway and enhancement of cellular trafficking were found to be accomplished in both neural cell lines after specific treatment with TAT-CNTF. Finally, the recombinant growth factor was successfully loaded on oxidized polyvinyl alcohol (PVA) scaffolds, and more efficiently released when polymer oxidation rate increased. Taken together, our results highlight that the TAT domain addiction to the protein sequence preserves CNTF specific neurotrophic activity in vitro, besides improving cellular uptake. Moreover, oxidized PVA could represent an ideal biomaterial for the development of nerve conduits loaded with the fusion protein to be delivered to the site of nerve injury. - Highlights: • TAT-CNTF is an optimized fusion protein that preserves neurotrophic activity. • In neural cell lines, TAT-CNTF triggers the activation of signal transduction. • Fast cellular uptake of TAT-CNTF was

In vitro assessment of TAT — Ciliary Neurotrophic Factor therapeutic potential for peripheral nerve regeneration

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Barbon, Silvia, E-mail: silvia.barbon@yahoo.it [Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Via Marzolo 5, 35131 Padua (Italy); Foundation for Biology and Regenerative Medicine, Tissue Engineering and Signaling (TES) ONLUS, Via De Sanctis 10, Caselle di Selvazzano Dentro, 35030 Padua (Italy); Stocco, Elena, E-mail: elena.stocco@gmail.com [Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Via Marzolo 5, 35131 Padua (Italy); Foundation for Biology and Regenerative Medicine, Tissue Engineering and Signaling (TES) ONLUS, Via De Sanctis 10, Caselle di Selvazzano Dentro, 35030 Padua (Italy); Negro, Alessandro, E-mail: alessandro.negro@unipd.it [Department of Biomedical Sciences, University of Padova, Via Colombo 3, 35121 Padua (Italy); Dalzoppo, Daniele, E-mail: daniele.dalzoppo@unipd.it [Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Via Marzolo 5, 35131 Padua (Italy); Borgio, Luca, E-mail: borgio.luca@gmail.com [Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Via Marzolo 5, 35131 Padua (Italy); Rajendran, Senthilkumar, E-mail: senthilstem@gmail.com [Department of Pharmaceutical and Pharmacological Sciences, University of Padua, Via Marzolo 5, 35131 Padua (Italy); Grandi, Francesca, E-mail: francesca.grandi7825@gmail.com [Department of Women' s and Children' s Health, Pediatric Surgery, University of Padua, Via Giustiniani 3, 35121 Padua (Italy); Porzionato, Andrea, E-mail: andrea.porzionato@unipd.it [Section of Human Anatomy, Department of Molecular Medicine, University of Padua, Via Gabelli 65, 35121 Padua (Italy); Macchi, Veronica, E-mail: veronica.macchi@unipd.it [Section of Human Anatomy, Department of Molecular Medicine, University of Padua, Via Gabelli 65, 35121 Padua (Italy); De Caro, Raffaele, E-mail: raffaele.decaro@unipd.it [Section of Human Anatomy, Department of Molecular Medicine, University of Padua, Via Gabelli 65, 35121 Padua (Italy); and others

2016-10-15

In regenerative neurobiology, Ciliary Neurotrophic Factor (CNTF) is raising high interest as a multifunctional neurocytokine, playing a key role in the regeneration of injured peripheral nerves. Despite its promising trophic and regulatory activity, its clinical application is limited by the onset of severe side effects, due to the lack of efficient intracellular trafficking after administration. In this study, recombinant CNTF linked to the transactivator transduction domain (TAT) was investigated in vitro and found to be an optimized fusion protein which preserves neurotrophic activity, besides enhancing cellular uptake for therapeutic advantage. Moreover, a compelling protein delivery method was defined, in the future perspective of improving nerve regeneration strategies. Following determination of TAT-CNTF molecular weight and concentration, its specific effect on neural SH-SY5Y and PC12 cultures was assessed. Cell proliferation assay demonstrated that the fusion protein triggers PC12 cell growth within 6 h of stimulation. At the same time, the activation of signal transduction pathway and enhancement of cellular trafficking were found to be accomplished in both neural cell lines after specific treatment with TAT-CNTF. Finally, the recombinant growth factor was successfully loaded on oxidized polyvinyl alcohol (PVA) scaffolds, and more efficiently released when polymer oxidation rate increased. Taken together, our results highlight that the TAT domain addiction to the protein sequence preserves CNTF specific neurotrophic activity in vitro, besides improving cellular uptake. Moreover, oxidized PVA could represent an ideal biomaterial for the development of nerve conduits loaded with the fusion protein to be delivered to the site of nerve injury. - Highlights: • TAT-CNTF is an optimized fusion protein that preserves neurotrophic activity. • In neural cell lines, TAT-CNTF triggers the activation of signal transduction. • Fast cellular uptake of TAT-CNTF was

Localization of functional receptor epitopes on the structure of ciliary neurotrophic factor indicates a conserved, function-related epitope topography among helical cytokines.

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Panayotatos, N; Radziejewska, E; Acheson, A; Somogyi, R; Thadani, A; Hendrickson, W A; McDonald, N Q

1995-06-09

By rational mutagenesis, receptor-specific functional analysis, and visualization of complex formation in solution, we identified individual amino acid side chains involved specifically in the interaction of ciliary neurotrophic factor (CNTF) with CNTFR alpha and not with the beta-components, gp130 and LIFR. In the crystal structure, the side chains of these residues, which are located in helix A, the AB loop, helix B, and helix D, are surface accessible and are clustered in space, thus constituting an epitope for CNTFR alpha. By the same analysis, a partial epitope for gp130 was also identified on the surface of helix A that faces away from the alpha-epitope. Superposition of the CNTF and growth hormone structures showed that the location of these epitopes on CNTF is analogous to the location of the first and second receptor epitopes on the surface of growth hormone. Further comparison with proposed binding sites for alpha- and beta-receptors on interleukin-6 and leukemia inhibitory factor indicated that this epitope topology is conserved among helical cytokines. In each case, epitope I is utilized by the specificity-conferring component, whereas epitopes II and III are used by accessory components. Thus, in addition to a common fold, helical cytokines share a conserved order of receptor epitopes that is function related.

Caracterización morfoanatómica comparativa entre Aloe vera (L. Burm. F., Aloe arborescens Mill., Aloe saponaria Haw. y Aloe ciliaris Haw. (Aloeaceae

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Stella Maris Carpano

Full Text Available Aloe vera (L. Burm. F. (= Aloe barbadensis Miller conhecida como "a planta da imortalidade" no antigo Egito é utilizada em fitoterapia como humectante, antibacteriana, antifúngica, antiviral e antioxidante. Com a finalidade de contribuir para identificação da droga vegetal, foram realizados estudos morfoanatômicos da folha. A utilização de plantas com características terapêuticas reconhecidas determina que, por analogia, popularmente sejam utilizadas com a mesma finalidade outras espécies do mesmo gênero. Isto é o que ocorre com três espécies de Aloe spp., que se cultivam ou crescem acidentalmente na Argentina: A. arborescens Mill., A. saponaria Haw. e A. ciliaris Haw. (Aloaceae. Estabeleceram-se os caracteres morfoanatômicos de diagnóstico por microscopia óptica e microscopia eletrônica de varredura (apresentam-se desenhos e fotomicrografias dos mesmos. Obtiveram-se valores numéricos dos elementos histológicos de diagnóstico: magnitudes lineares e proporcionais. Realizaram-se reações histoquímicas de identificação dos princípios ativos para sua localização "in situ".

Non‐diluted seawater enhances nasal ciliary beat frequency and wound repair speed compared to diluted seawater and normal saline

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Bonnomet, Arnaud; Luczka, Emilie; Coraux, Christelle

2016-01-01

Background The regulation of mucociliary clearance is a key part of the defense mechanisms developed by the airway epithelium. If a high aggregate quality of evidence shows the clinical effectiveness of nasal irrigation, there is a lack of studies showing the intrinsic role of the different irrigation solutions allowing such results. This study investigated the impact of solutions with different pH and ionic compositions, eg, normal saline, non‐diluted seawater and diluted seawater, on nasal mucosa functional parameters. Methods For this randomized, controlled, blinded, in vitro study, we used airway epithelial cells obtained from 13 nasal polyps explants to measure ciliary beat frequency (CBF) and epithelial wound repair speed (WRS) in response to 3 isotonic nasal irrigation solutions: (1) normal saline 0.9%; (2) non‐diluted seawater (Physiomer®); and (3) 30% diluted seawater (Stérimar). The results were compared to control (cell culture medium). Results Non‐diluted seawater enhanced the CBF and the WRS when compared to diluted seawater and to normal saline. When compared to the control, it significantly enhanced CBF and slightly, though nonsignificantly, improved the WRS. Interestingly, normal saline markedly reduced the number of epithelial cells and ciliated cells when compared to the control condition. Conclusion Our results suggest that the physicochemical features of the nasal wash solution is important because it determines the optimal conditions to enhance CBF and epithelial WRS thus preserving the respiratory mucosa in pathological conditions. Non‐diluted seawater obtains the best results on CBF and WRS vs normal saline showing a deleterious effect on epithelial cell function. PMID:27101776

[Ultrastructure of epithelium and ciliary receptors in the parasitic turbellarian Urastoma cyprinae (Turbellaria, "Prolecithophora") and position of the species within Platyhelminthes].

Science.gov (United States)

Kornakova, E E

2002-01-01

be the preadaptation to the endoparasitic mode of life in Fecampiida. The differencies in ultrastructure of epithelium in U. cyprinae from the White Sea and from Mediterranean Sea (Noury-Sraïri e. a., 1990) may be explained by the differences in the method of fixation or by the parasitizing the another host--the mollusk Mytilus galloprovincialis. The ciliary receptors of five types were revealed in U. cyprinae (fig. 3, e, [symbol: see text]; 4; 5; 6). They differ in the shape and length of the ciliary rootlets and in the content of the nerve processes. All receptors lack of the real collars typical for the receptors of Neodermata. Urastoma is most close to the Neodermata amond parasitic turbellarians studied thus far, and the absence of collars in receptors of this species testifies that the collars are the veritable synapomorphy of the Neodermata. The diversity in the ultrastructure and possible functions of receptors correspond to the complicated adaptations of this species. The modern molecular data as well as the ultrastructural evidence attest that parasitic turbellarians of the genera Urastoma, Genostoma and Ichthyophaga are relatives and cannot be included in any turbellarian order known. Therefore Urastoma, Genostoma and Ichthyophaga have been erected in the separate order Urastomida ord. nov. The diagnosis of the new order is given.

Pigment dispersion glaucoma induced by the chafing effect of intraocular lens haptics in Asian eyes.

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Hong, Ying; Sun, Yan-Xiu; Qi, Hong; Zhou, Ji-Chao; Hao, Yan-Sheng

2013-03-01

To study the possible mechanism and treatment for pigment dispersion glaucoma (PDG) caused by single-piece acrylic (SPA) intraocular lens (IOL) ciliary sulcus fixation in Asian eyes. Patients referred for PDG caused by SPA IOL ciliary sulcus fixation to our hospital from April 2005 to June 2011 were included. The patients' general information, IOL type, interval between initial surgery and PDG occurrence, examination findings, antiglaucoma medicine regimen and surgical interventions were recorded. In total, six eyes from five Chinese patients were included in this study. The intraocular pressure (IOP) increased 19-30 days after cataract surgery and was not satisfactorily controlled with antiglaucoma medication. Dense pigmentation was deposited on the IOLs and on the anterior chamber angle. IOL haptic chafing was noted on the rear iris surface. IOL repositioning in the capsular bag was performed in three eyes and was combined with trabeculectomy in two eyes with progressive glaucoma. An IOL exchange with three-piece IOL ciliary sulcus fixation was performed in the other three eyes. Scanning electron microscopy of the explanted IOLs demonstrated a rough edge on the IOL haptics. SPA IOLs were not suitable for ciliary sulcus fixation. The chafing effect of the IOL haptics on the posterior iris pigment epithelium could induce PDG in Asian eyes. IOLs should be positioned in the capsular bag or a three-piece IOL should be used instead.

EVALUATION OF IRIDOCILIARY AND LENTICULAR ELASTICITY USING SHEAR-WAVE ELASTOGRAPHY IN RABBIT EYES

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Efstathios T. Detorakis

2014-01-01

Full Text Available Introduction: A previous study has employed shear-wave ultrasound elastographic imaging to assess corneal rigidity in an ex-vivo porcine eye model. This study employs the same modality in vivo in a rabbit eye model in order to assess lens, ciliary body and total ocular rigidity changes following the instillation of atropine and pilocarpine. Methods: Ten non-pigmented female rabbits were examined. Measurements of the lens, ciliary body and total ocular rigidity as well as lens thickness and anterior chamber depth were taken with the Aixplorer system (SuperSonic Imagine, Aix-en-Provence, France with the SuperLinear™ SL 15-4 transducer in both eyes at baseline as well as after pilocarpine and atropine instillation. The IOP was also measured with the TonoPen tonometer. Results: Changes in rigidity in the examined areas following atropine instillation were statistically not significant. Ciliary body rigidity was significantly increased whereas lens and total ocular rigidity were significantly reduced following pilocarpine instillation. The decrease in lens rigidity following pilocarpine was significantly associated with the respective increase in ciliary body rigidity. Conclusions: Shear-wave ultrasound elastography can detect in vivo rigidity changes in the anterior segment of the rabbit eye model and may potentially be applied in human eyes, providing useful clinical information on conditions in which rigidity changes play an important role, such as glaucoma, pseudoexfoliation syndrome or presbyopia.

Evaluation of iridociliary and lenticular elasticity using shear-wave elastography in rabbit eyes.

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Detorakis, Efstathios T; Drakonaki, Eleni E; Ginis, Harilaos; Karyotakis, Nikolaos; Pallikaris, Ioannis G

2014-01-01

A previous study has employed shear-wave ultrasound elastographic imaging to assess corneal rigidity in an ex-vivo porcine eye model. This study employs the same modality in vivo in a rabbit eye model in order to assess lens, ciliary body and total ocular rigidity changes following the instillation of atropine and pilocarpine. Ten non-pigmented female rabbits were examined. Measurements of the lens, ciliary body and total ocular rigidity as well as lens thickness and anterior chamber depth were taken with the Aixplorer system (SuperSonic Imagine, Aix-en-Provence, France) with the SuperLinear™ SL 15-4 transducer in both eyes at baseline as well as after pilocarpine and atropine instillation. The IOP was also measured with the TonoPen tonometer. Changes in rigidity in the examined areas following atropine instillation were statistically not significant. Ciliary body rigidity was significantly increased whereas lens and total ocular rigidity were significantly reduced following pilocarpine instillation. The decrease in lens rigidity following pilocarpine was significantly associated with the respective increase in ciliary body rigidity. Shear-wave ultrasound elastography can detect in vivo rigidity changes in the anterior segment of the rabbit eye model and may potentially be applied in human eyes, providing useful clinical information on conditions in which rigidity changes play an important role, such as glaucoma, pseudoexfoliation syndrome or presbyopia.

Lung structure and function similarities between primary ciliary dyskinesia and mild cystic fibrosis: a pilot study.

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Maglione, Marco; Montella, Silvia; Mollica, Carmine; Carnovale, Vincenzo; Iacotucci, Paola; De Gregorio, Fabiola; Tosco, Antonella; Cervasio, Mariarosaria; Raia, Valeria; Santamaria, Francesca

2017-04-12

Primary ciliary dyskinesia (PCD) and cystic fibrosis (CF) are increasingly compared. There are no chest magnetic resonance imaging (MRI) comparative studies of PCD and CF. We assessed clinical, functional, microbiological and MRI findings in PCD and mild CF patients in order to evaluate different expression of lung disease. Twenty PCD (15.1 years) and 20 CF subjects with mild respiratory impairment (16 years, 70% with pancreatic insufficiency) underwent MRI, spirometry, and sputum cultures when clinically stable. MRI was scored using the modified Helbich system. PCD was diagnosed later than CF (9.9 versus 0.6 years, p = 0.03), despite earlier symptoms (0.1 versus 0.6 years, p = 0.02). In the year preceding the study, patients from both groups underwent two systemic antibiotic courses (p = 0.48). MRI total scores were 11.6 ± 0.7 and 9.1 ± 1 in PCD and CF, respectively. FEV 1 and FVC Z-scores were -1.75 (range, -4.6-0.7) and -0.6 (-3.9-1.8) in PCD, and -0.9 (range, -5.4-2.3) and -0.3 (-3.4-2.5) in CF, respectively. No difference was found between lung function or structure, despite a higher MRI subscore of collapse/consolidation in PCD versus CF (1.6 ± 0.1 and 0.6 ± 0.2, p < 0.001). These findings were confirmed after data-control for diagnostic delay. Pseudomonas aeruginosa and Staphylococcus aureus were more frequent in CF than in PCD (p = 0.05 and p = 0.003, respectively). MRI is a valuable radiation-free tool for comparative PCD and CF lung disease assessment. Patients with PCD may exhibit similar MRI and lung function changes as CF subjects with mild pulmonary disease. Delay in PCD diagnosis is unlikely the only determinant of similarities.

Paramecium BBS genes are key to presence of channels in Cilia

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Valentine Megan

2012-09-01

Full Text Available Abstract Background Changes in genes coding for ciliary proteins contribute to complex human syndromes called ciliopathies, such as Bardet-Biedl Syndrome (BBS. We used the model organism Paramecium to focus on ciliary ion channels that affect the beat form and sensory function of motile cilia and evaluate the effects of perturbing BBS proteins on these channels. Methods We used immunoprecipitations and mass spectrometry to explore whether Paramecium proteins interact as in mammalian cells. We used RNA interference (RNAi and swimming behavior assays to examine the effects of BBS depletion on ciliary ion channels that control ciliary beating. Combining RNA interference and epitope tagging, we examined the effects of BBS depletion of BBS 7, 8 and 9 on the location of three channels and a chemoreceptor in cilia. Results We found 10 orthologs of 8 BBS genes in P. tetraurelia. BBS1, 2, 4, 5, 7, 8 and 9 co-immunoprecipitate. While RNAi reduction of BBS 7 and 9 gene products caused loss and shortening of cilia, RNAi for all BBS genes except BBS2 affected patterns of ciliary motility that are governed by ciliary ion channels. Swimming behavior assays pointed to loss of ciliary K+ channel function. Combining RNAi and epitope tagged ciliary proteins we demonstrated that a calcium activated K+ channel was no longer located in the cilia upon depletion of BBS 7, 8 or 9, consistent with the cells’ swimming behavior. The TRPP channel PKD2 was also lost from the cilia. In contrast, the ciliary voltage gated calcium channel was unaffected by BBS depletion, consistent with behavioral assays. The ciliary location of a chemoreceptor for folate was similarly unperturbed by the depletion of BBS 7, 8 or 9. Conclusions The co-immunoprecipitation of BBS 1,2,4,5,7,8, and 9 suggests a complex of BBS proteins. RNAi for BBS 7, 8 or 9 gene products causes the selective loss of K+ and PKD2 channels from the cilia while the critical voltage gated calcium channel and a

Existence of multiple receptors in single neurons: responses of single bullfrog olfactory neurons to many cAMP-dependent and independent odorants.

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Kashiwayanagi, M; Shimano, K; Kurihara, K

1996-11-04

The responses of single bullfrog olfactory neurons to various odorants were measured with the whole-cell patch clamp which offers direct information on cellular events and with the ciliary recording technique to obtain stable quantitative data from many neurons. A large portion of single olfactory neurons (about 64% and 79% in the whole-cell recording and in the ciliary recording, respectively) responded to many odorants with quite diverse molecular structures, including both odorants previously indicated to be cAMP-dependent (increasing) and independent odorants. One odorant elicited a response in many cells; e.g. hedione and citralva elicited the response in 100% and 92% of total neurons examined with the ciliary recording technique. To confirm that a single neuron carries different receptors or transduction pathways, the cross-adaptation technique was applied to single neurons. Application of hedione to a single neuron after desensitization of the current in response to lyral or citralva induced an inward current with a similar magnitude to that applied alone. It was suggested that most single olfactory neurons carry multiple receptors and at least dual transduction pathways.

Primary ciliary dyskinesia in the paediatric population: range and severity of radiological findings in a cohort of patients receiving tertiary care

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Jain, K. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom); Padley, S.P.G. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom)], E-mail: s.padley@ic.ac.uk; Goldstraw, E.J.; Kidd, S.J. [Department of Radiology, Royal Brompton and Harefield NHS Trust, London (United Kingdom); Hogg, C.; Biggart, E.; Bush, A. [Department of Paediatric Respiratory Medicine, Royal Brompton and Harefield NHS Trust, London (United Kingdom)

2007-10-15

Aim: To investigate the clinical range and severity of radiological findings in a cohort of patients with primary ciliary dyskinesia (PCD) receiving tertiary care. Materials and methods: The case notes and clinical test results of 89 children attending the paediatric respiratory disease clinic at our institution were retrospectively analysed. Demographic details including age at diagnosis and common presenting signs and symptoms were studied. Results of chest radiographs, microscopy, and high-resolution computed tomography (HRCT) for quantification of lung damage were analysed. Results: In a cohort of 89 children with PCD, a presentation chest radiograph was available in 62% of patients (n = 55), with all but one demonstrating changes of bronchial wall thickening. HRCT of the lungs, available in 26 patients, were scored using the system described by Brody et al. analysing five specific features of lung disease, including bronchiectasis, mucus plugging, peribronchial thickening, parenchymal changes of consolidation, and ground-glass density, and focal air-trapping in each lobe. Peribronchial thickening was observed using HRCT in 25 patients, while 20 patients had bronchiectasis. Severity scores were highest for the middle and the lingular lobes. Conclusion: The radiographic findings of the largest reported cohort of patients with PCD are presented, with associated clinical findings. Dextrocardia remains the commonest finding on chest radiography. HRCT demonstrates peribronchial thickening and bronchiectasis, which is most marked in the lower zones. Radiological scoring techniques developed for assessment of cystic fibrosis can also be applied for the assessment of disease severity in this patient population.

Influence of Maternal Undernutrition and Overfeeding on Cardiac Ciliary Neurotrophic Factor Receptor and Ventricular Size in Fetal Sheep

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Dong, Feng; Ford, Stephen P.; Nijland, Mark J.; Nathanielsz, Peter W.; Ren, Jun

2008-01-01

Intrauterine nutrition status is reported to correlate with risk of cardiovascular diseases in adulthood. Either under- or over-nutrition during early to mid gestation contributes to altered fetal growth and ventricular geometry. This study was designed to examine myocardial expression of ciliary neurotrophic factor receptor α (CTNFRα) and its down-stream mediator signal transducer and activator of transcription 3 (STAT3) on maternal under- or over-nutrition-induced changes in fetal heart weight. Multiparous ewes were fed with 50% (nutrient-restricted, NR), 100% (control) or 150% (overfed, OF) of NRC requirements from 28 to 78 days of gestation (dG; Term 148 dG). Ewes were euthanized on day 78, and the gravid uteri and fetuses recovered. Ventricular protein expression of CTNFRα, STAT3, phosphorylated STAT3, insulin-like growth factor I receptor (IGF-1R) and IGF binding protein 3 (IGFBP3) were quantitated using western blot. Plasma cortisol levels were higher in both NR and OF fetuses whereas plasma IGF-1 levels were lower and higher, in NR and OF fetuses. Fetal weights were reduced by 29.9% in NR ewes and were increased by 22.2% in fetuses from OF ewes compared to control group. Nutrient restriction did not affect fetal heart or ventricular weights whereas overfeeding increased heart and ventricular weights. Protein expression of CTNFRα in fetal ventricular tissue was reduced in OF group whereas STAT3 and pSTAT3 levels were reduced in both NR and OF groups. Expression of IGF-1R and IGFBP3 was unaffected in either NR or OF group. These data suggested that compared with maternal undernutrition, intrauterine overfeeding during early to mid gestation is associated with increases fetal blood concentrations of cortisol and IGF-1 in association with ventricular hypertrophy where reduced expression of CNTFRα and STAT3 may play a role. PMID:17869083

Cochliotoxin, a Dihydropyranopyran-4,5-dione, and Its Analogues Produced by Cochliobolus australiensis Display Phytotoxic Activity against Buffelgrass (Cenchrus ciliaris).

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Masi, Marco; Meyer, Susan; Clement, Suzette; Cimmino, Alessio; Cristofaro, Massimo; Evidente, Antonio

2017-05-26

Buffelgrass (Pennisetum ciliare or Cenchrus ciliaris) is a perennial grass that has become highly invasive in the Sonoran Desert of southern Arizona. In the search for novel control strategies against this weed, strains of the foliar fungal pathogen Cochliobolus australiensis from buffelgrass have been screened for their ability to produce phytotoxic metabolites that could potentially be used as natural herbicides in an integrated pest management strategy. A new phytotoxin, named cochliotoxin, was isolated from liquid culture of this fungus together with radicinin, radicinol, and their 3-epimers. Cochliotoxin was characterized, essentially by spectroscopic methods, as 3-hydroxy-2-methyl-7-(3-methyloxiranyl)-2,3-dihydropyrano[4,3-b]pyran-4,5-dione. Its relative stereochemistry was assigned by 1 H NMR techniques, while the absolute configuration (2S,3S) was determined applying the advanced Mosher's method by esterification of its hydroxy group at C-3. When bioassayed in a buffelgrass coleoptile elongation test and by leaf puncture bioassay against the host weed and two nontarget grasses, cochliotoxin showed strong phytotoxicity. In the same tests, radicinin and 3-epi-radicinin also showed phytotoxic activity, while radicinol and 3-epi-radicinol were largely inactive. All five compounds were more active in leaf puncture bioassays on buffelgrass than on the nontarget grass tanglehead (Heteropogon contortus), while the nontarget grass Arizona cottontop (Digitaria californica) was more sensitive to radicinin and 3-epi-radicinin. Cochliotoxin at low concentration was significantly more active on buffelgrass than on either native grass, but the difference was small.

Non-diluted seawater enhances nasal ciliary beat frequency and wound repair speed compared to diluted seawater and normal saline.

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Bonnomet, Arnaud; Luczka, Emilie; Coraux, Christelle; de Gabory, Ludovic

2016-10-01

The regulation of mucociliary clearance is a key part of the defense mechanisms developed by the airway epithelium. If a high aggregate quality of evidence shows the clinical effectiveness of nasal irrigation, there is a lack of studies showing the intrinsic role of the different irrigation solutions allowing such results. This study investigated the impact of solutions with different pH and ionic compositions, eg, normal saline, non-diluted seawater and diluted seawater, on nasal mucosa functional parameters. For this randomized, controlled, blinded, in vitro study, we used airway epithelial cells obtained from 13 nasal polyps explants to measure ciliary beat frequency (CBF) and epithelial wound repair speed (WRS) in response to 3 isotonic nasal irrigation solutions: (1) normal saline 0.9%; (2) non-diluted seawater (Physiomer®); and (3) 30% diluted seawater (Stérimar). The results were compared to control (cell culture medium). Non-diluted seawater enhanced the CBF and the WRS when compared to diluted seawater and to normal saline. When compared to the control, it significantly enhanced CBF and slightly, though nonsignificantly, improved the WRS. Interestingly, normal saline markedly reduced the number of epithelial cells and ciliated cells when compared to the control condition. Our results suggest that the physicochemical features of the nasal wash solution is important because it determines the optimal conditions to enhance CBF and epithelial WRS thus preserving the respiratory mucosa in pathological conditions. Non-diluted seawater obtains the best results on CBF and WRS vs normal saline showing a deleterious effect on epithelial cell function. © 2016 The Authors International Forum of Allergy & Rhinology, published by ARSAAOA, LLC.

Forecasting an invasive species’ distribution with global distribution data, local data, and physiological information

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Jarnevich, Catherine S.; Young, Nicholas E.; Talbert, Marian; Talbert, Colin

2018-01-01

Understanding invasive species distributions and potential invasions often requires broad‐scale information on the environmental tolerances of the species. Further, resource managers are often faced with knowing these broad‐scale relationships as well as nuanced environmental factors related to their landscape that influence where an invasive species occurs and potentially could occur. Using invasive buffelgrass (Cenchrus ciliaris), we developed global models and local models for Saguaro National Park, Arizona, USA, based on location records and literature on physiological tolerances to environmental factors to investigate whether environmental relationships of a species at a global scale are also important at local scales. In addition to correlative models with five commonly used algorithms, we also developed a model using a priori user‐defined relationships between occurrence and environmental characteristics based on a literature review. All correlative models at both scales performed well based on statistical evaluations. The user‐defined curves closely matched those produced by the correlative models, indicating that the correlative models may be capturing mechanisms driving the distribution of buffelgrass. Given climate projections for the region, both global and local models indicate that conditions at Saguaro National Park may become more suitable for buffelgrass. Combining global and local data with correlative models and physiological information provided a holistic approach to forecasting invasive species distributions.

Adenoid ciliostimulation in children with chronic otitis media.

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Andreoli, Steven M; Schlosser, Rodney J; Wang, Ling-Feng; Mulligan, Ryan M; Discolo, Christopher M; White, David R

2013-01-01

Adenoid hypertrophy and chronic adenoiditis are associated with an increased incidence of chronic otitis media. This study intends to determine the relationship between chronic otitis media and dynamic ciliary beat frequency in children undergoing adenoidectomy. Prospective, controlled study. Pediatric tertiary care hospital. Children undergoing adenoidectomy were enrolled. Patients were stratified according to their indication for surgery, including adenotonsillar hypertrophy with obstructive sleep apnea, chronic otitis media with effusion, or recurrent episodes of acute otitis media. Adenoids were harvested using the curette. Tissue was sectioned and allowed to equilibrate in basal media for 24 hours. Cilia-bearing tissue was then stimulated using isoproterenol or methacholine. Ciliary beat frequency was serially reordered and analyzed using the Sisson-Ammons Video Analysis software program. Baseline ciliary beat frequency was similar in all groups (N = 47, total). Using isoproterenol, children with chronic otitis media with effusion demonstrated a blunted dynamic ciliary response at 2 and 3 hours relative to control (P = .0176 and P = .0282). Methacholine-stimulated ciliary beat frequency was not different between each group. At 2 and 3 hours following isoproterenol stimulation, there was a significant blunting of dynamic ciliary beat frequency in children with chronic otitis media with effusion. This ciliary dysfunction may provide a physiological explanation related to chronic adenoiditis in children with chronic otitis media.

CEP128 Localizes to the Subdistal Appendages of the Mother Centriole and Regulates TGF-β/BMP Signaling at the Primary Cilium

DEFF Research Database (Denmark)

Mönnich, Maren; Borgeskov, Louise; Breslin, Loretta

2018-01-01

The centrosome is the main microtubule-organizing center in animal cells and comprises a mother and daughter centriole surrounded by pericentriolar material. During formation of primary cilia, the mother centriole transforms into a basal body that templates the ciliary axoneme. Ciliogenesis depends...... on mother centriole-specific distal appendages, whereas the role of subdistal appendages in ciliary function is unclear. Here, we identify CEP128 as a centriole subdistal appendage protein required for regulating ciliary signaling. Loss of CEP128 did not grossly affect centrosomal or ciliary structure...... but caused impaired transforming growth factor-β/bone morphogenetic protein (TGF-β/BMP) signaling in zebrafish and at the primary cilium in cultured mammalian cells. This phenotype is likely the result of defective vesicle trafficking at the cilium as ciliary localization of RAB11 was impaired upon loss...

Effects of Resistance Training on Muscle Strength, Endurance, and Motor Unit According to Ciliary Neurotrophic Factor Polymorphism in Male College Students

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Ae-Rim Hong, Sang-Min Hong, Yun-A Shin

2014-09-01

Full Text Available Changes in muscle mass and strength across the adult age span are variable and related to the ciliary neurotrophic factor (CNTF genotype. In particular, a single CNTF haplotype (1357 G→A is important for neuronal and muscular developments and may be associated with muscle strength response to resistance training. We examined whether CNTF genotype differentially influences the effect of resistance training on neuromuscular improvement in male college students. Resistance training of the upper extremities comprised 3 sets at 75%–85% intensity per 1 repetition maximum, 3 times a week, for a total of 8 weeks. We measured isokinetic muscle function of the elbow joint with regard to strength (60°/s and endurance (180°/s by using an isokinetic dynamometer. The biceps brachii (BB and brachioradialis muscles were studied using surface electromyography with spike-triggered averaging to assess surface-detected motor unit potential (SMUP area. After resistance training, the SMUP of the BB increased significantly at 60°/s (p < 0.05, but no difference in the CNTF genotype was observed. The SMUP of the BB at 180°/s increased significantly in the GG/AA genotype group compared with that in the GA genotype group (p < 0.05. The average power of the elbow flexor at 180°/s increased significantly after resistance training (p < 0.05, but again, no difference in the CNTF genotype was observed. Thus, improvements in muscle strength and endurance may have resulted directly from resistance training rather than from genetic factors related to nerves in muscle tissue.

Vicks VapoRub induces mucin secretion, decreases ciliary beat frequency, and increases tracheal mucus transport in the ferret trachea.

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Abanses, Juan Carlos; Arima, Shinobu; Rubin, Bruce K

2009-01-01

Vicks VapoRub (VVR) [Proctor and Gamble; Cincinnati, OH] is often used to relieve symptoms of chest congestion. We cared for a toddler in whom severe respiratory distress developed after VVR was applied directly under her nose. We hypothesized that VVR induced inflammation and adversely affected mucociliary function, and tested this hypothesis in an animal model of airway inflammation. [1] Trachea specimens excised from 15 healthy ferrets were incubated in culture plates lined with 200 mg of VVR, and the mucin secretion was compared to those from controls without VVR. Tracheal mucociliary transport velocity (MCTV) was measured by timing the movement of 4 microL of mucus across the trachea. Ciliary beat frequency (CBF) was measured using video microscopy. [2] Anesthetized and intubated ferrets inhaled a placebo or VVR that was placed at the proximal end of the endotracheal tube. We evaluated both healthy ferrets and animals in which we first induced tracheal inflammation with bacterial endotoxin (a lipopolysaccharide [LPS]). Mucin secretion was measured using an enzyme-linked lectin assay, and lung water was measured by wet/dry weight ratios. [1] Mucin secretion was increased by 63% over the controls in the VVR in vitro group (p < 0.01). CBF was decreased by 35% (p < 0.05) in the VVR group. [2] Neither LPS nor VVR increased lung water, but LPS decreased MCTV in both normal airways (31%) and VVR-exposed airways (30%; p = 0.03), and VVR increased MCTV by 34% in LPS-inflamed airways (p = 0.002). VVR stimulates mucin secretion and MCTV in the LPS-inflamed ferret airway. This set of findings is similar to the acute inflammatory stimulation observed with exposure to irritants, and may lead to mucus obstruction of small airways and increased nasal resistance.

Patient-specific three-dimensional explant spheroids derived from human nasal airway epithelium

DEFF Research Database (Denmark)

Marthin, June Kehlet; Stevens, Elizabeth Munkebjerg; Larsen, Lars Allan

2017-01-01

BACKGROUND: Three-dimensional explant spheroid formation is an ex vivo technique previously used in studies of airway epithelial ion and water transport. Explanted cells and sheets of nasal epithelium form fully differentiated spheroids enclosing a partly fluid-filled lumen with the ciliated apical...... surface facing the outside and accessible for analysis of ciliary function. METHODS: We performed a two-group comparison study of ciliary beat pattern and ciliary beat frequency in spheroids derived from nasal airway epithelium in patients with primary ciliary dyskinesia (PCD) and in healthy controls...... in the investigation of pathophysiological aspects and drug effects in human nasal airway epithelium....

Case report: imaging and treatment of ophthalmic manifestations in oculodentodigital dysplasia.

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Mosaed, Sameh; Jacobsen, Bradley H; Lin, Ken Young

2016-01-07

Diagnostic and surgical management of severe chronic angle- closure glaucoma secondary to ciliary body cysts can be difficult to manage in a patient with oculodentodigital dysplasia. A 6-year old girl with oculodentodigital dysplasia, with progressive chronic angle- closure glaucoma secondary to ciliary body cysts presented to our clinic. The initial examination revealed counting fingers vision in the left eye. Intraocular pressure (IOP), as assessed by tonopen, was 31 mm Hg. Ultrasound biomicroscopy revealed ciliary body cysts in the left eye, and gonioscopy confirmed chronic angle closure. A tube shunt was placed to control the elevated IOP. A year after her tube shunt placement in the left eye, ultrasound biomiscropy was performed on her right eye and showed no ciliary body cysts. Gonioscopy in the right eye revealed an open angle to the ciliary body band. Subsequent serial gonioscopy every 3 months showed gradual narrowing of the right eye angle and finally three-and-a-half years after tube placement of the left eye, her right eye IOP became uncontrolled with medications alone and a tube shunt was similarly placed in the right eye. Intraoperative ultrasound biomicroscopy performed at the time of the right eye tube shunt revealed extensive ciliary body cysts in the right eye. Her IOP in both eyes have been well controlled since the placement of tube shunts. This is one of the first reported cases of severe chronic angle- closure glaucoma secondary to ciliary body cysts in a patient with oculodentodigital dysplasia. We believe that early screening for ciliary body cysts is important in patients with oculodentodigital dysplasia.

Loss of Centrobin Enables Daughter Centrioles to Form Sensory Cilia in Drosophila.

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Gottardo, Marco; Pollarolo, Giulia; Llamazares, Salud; Reina, Jose; Riparbelli, Maria G; Callaini, Giuliano; Gonzalez, Cayetano

2015-08-31

Sensory cilia are organelles that convey information to the cell from the extracellular environment. In vertebrates, ciliary dysfunction results in ciliopathies that in humans comprise a wide spectrum of developmental disorders. In Drosophila, sensory cilia are found only in the neurons of type I sensory organs, but ciliary dysfunction also has dramatic consequences in this organism because it impairs the mechanosensory properties of bristles and chaetae and leads to uncoordination, a crippling condition that causes lethality shortly after eclosion. The cilium is defined by the ciliary membrane, a protrusion of the cell membrane that envelops the core structure known as the axoneme, a microtubule array that extends along the cilium from the basal body. In vertebrates, basal body function requires centriolar distal and subdistal appendages and satellites. Because these structures are acquired through centriole maturation, only mother centrioles can serve as basal bodies. Here, we show that although centriole maturity traits are lacking in Drosophila, basal body fate is reserved to mother centrioles in Drosophila type I neurons. Moreover, we show that depletion of the daughter-centriole-specific protein Centrobin (CNB) enables daughter centrioles to dock on the cell membrane and to template an ectopic axoneme that, although structurally defective, protrudes out of the cell and is enveloped by a ciliary membrane. Conversely, basal body capability is inhibited in mother centrioles modified to carry CNB. These results reveal the crucial role of CNB in regulating basal body function in Drosophila ciliated sensory organs. Copyright © 2015 Elsevier Ltd. All rights reserved.

[Lens exchange for subluxation of posterior chamber lenses implanted in the capsular bag or in the ciliary sulcus].

Science.gov (United States)

Stürmer, J

2013-04-01

There are an increasing number of patients with decreased vision due to dislocated posterior chamber lenses, with pseudoexfoliation being the main risk factor. Various techniques for refixation of the subluxated posterior chamber IOL have been described. Experience with our technique of IOL-explantation, anterior vitrectomy and implantation of an Artisan anterior chamber lens are presented. In a retrospective study design all lens exchanges with implantation of an Artisan anterior chamber lens performed between 2003 and 2012 are analyzed. The study included 65 eyes of 61 patients (age 79.6 ± 9.2 years: 43-98). The majority of eyes (46/65; 70.8%) had Sundown Syndrome (late in-the-bag intraocular lens dislocation), in 19 eyes the posterior chamber lens was implanted primary or secondary into the ciliary sulcus. In the 46 eyes with Sundown Syndrome cataract surgery with implantation of a posterior chamber lens in the capsular bag was performed 7.4 ± 3.7 (1-22) years before subluxation within the bag. Pseudoexfoliation was the main risk factor in 42/46 (91.2%) of these eyes. A capsular tension ring (CTR) was implanted during cataract surgery in 34/46 (73.9%) eyes. The 34 IOLs with a CTR luxated significantly earlier (p IOLs without a CTR (6.6 ± 3.6 years; median 5.8 vs. 9.4 ± 3.1 years; median 9.2). The average visual gain was 0.2 logMAR in the group of luxated capsular bag lenses and 0.12 logMAR in the group of luxated sulcus lenses. Postoperative IOP decompensation was seen in 17/65 (26.2%) eyes (requiring IOP-lowering surgery in 8 eyes), 7 eyes developed corneal decompensation, 5 eyes had central retinal vein occlusion and one eye developed postoperative endophthalmitis. Lens exchange with implantation of an Artisan anterior chamber lens has become a routine procedure to improve vision in patients with subluxated IOLs. Postoperative IOP decompensation and vascular problems are the major complications. Georg Thieme Verlag KG Stuttgart · New York.

Pyriculins A and B, two monosubstituted hex-4-ene-2,3-diols and other phytotoxic metabolites produced by Pyricularia grisea isolated from buffelgrass (Cenchrus ciliaris).

Science.gov (United States)

Masi, Marco; Meyer, Susan; Górecki, Marcin; Mandoli, Alessandro; Di Bari, Lorenzo; Pescitelli, Gennaro; Cimmino, Alessio; Cristofaro, Massimo; Clement, Suzette; Evidente, Antonio

2017-11-01

Pyricularia grisea has been identified as a foliar pathogen on buffelgrass (Cenchrus ciliaris) in North America and was studied as a potential source of phytotoxins for buffelgrass control. Two monosubstituted hex-4-ene-2,3-diols, named pyriculins A and B, were isolated from its culture filtrate organic extract together with (10S,11S)-(-)-epipyriculol, trans-3,4-dihydro-3,4,8-trihydroxy-1(2H)-napthalenone, and (4S)-(+)-isosclerone. Pyriculins A and B were characterized by spectroscopic (essentially nuclear magnetic resonance [NMR], High-resolution electrospray ionization mass spectrometry [HRESIMS]) and chemical methods such as (4E)-1-(4-hydroxy-1,3-dihydroisobenzofuran-1-yl)hex-4-ene-2,3-diols. The relative and absolute configuration of these compounds was determined by a combination of spectroscopic (NMR, electronic circular dichroism [ECD]) and computational tools. When bioassayed in a buffelgrass coleoptile and radicle elongation test, (10S,11S)-(-)-epipyriculol proved to be the most toxic compound. Seed germination was much reduced and slowed with respect to the control and radicles failed to elongate. All five compounds delayed germination, but only (10S,11S)-(-)-epipyriculol was able to prevent radicle development of buffelgrass seedlings. It had no effect on coleoptile elongation, while the other four compounds caused significantly increased coleoptile development relative to the control. © 2017 Wiley Periodicals, Inc.

Loss of Arf4 causes severe degeneration of the exocrine pancreas but not cystic kidney disease or retinal degeneration.

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Jillian N Pearring

2017-04-01

Full Text Available Arf4 is proposed to be a critical regulator of membrane protein trafficking in early secretory pathway. More recently, Arf4 was also implicated in regulating ciliary trafficking, however, this has not been comprehensively tested in vivo. To directly address Arf4's role in ciliary transport, we deleted Arf4 specifically in either rod photoreceptor cells, kidney, or globally during the early postnatal period. Arf4 deletion in photoreceptors did not cause protein mislocalization or retinal degeneration, as expected if Arf4 played a role in protein transport to the ciliary outer segment. Likewise, Arf4 deletion in kidney did not cause cystic disease, as expected if Arf4 were involved in general ciliary trafficking. In contrast, global Arf4 deletion in the early postnatal period resulted in growth restriction, severe pancreatic degeneration and early death. These findings are consistent with Arf4 playing a critical role in endomembrane trafficking, particularly in the pancreas, but not in ciliary function.

Emerging ciliopathies: are respiratory cilia compromised in Usher syndrome?

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Piatti, G; De Santi, M M; Brogi, M; Castorina, P; Ambrosetti, U

2014-01-01

Usher syndrome is a ciliopathy involving photoreceptors and cochlear hair cells (sensory cilia): since sensory and motor ciliopathies can overlap, we analysed the respiratory cilia (motile) in 17 patients affected by Usher syndrome and 18 healthy control subject. We studied the mucociliary transport time with the saccharine test, ciliary motility and ultrastructure of respiratory cilia obtained by nasal brushing; we also recorded the classical respiratory function values by spirometry. All enrolled subjects showed normal respiratory function values. The mean mucociliary transport time with saccharine was 22.33 ± 17.96 min, which is in the range of normal values. The mean ciliary beat frequency of all subjects was 8.81 ± 2.18 Hz, which is a value approaching the lower physiological limit. None of the classical ciliary alterations characterizing the "ciliary primary dyskinesia" was detected, although two patients showed alterations in number and arrangement of peripheral microtubules and one patient had abnormal ciliary roots. Respiratory cilia in Usher patients don't seem to have evident ultrastructural alterations, as expected, but the fact that the ciliary motility appeared slightly reduced could emphasize that a rigid distinction between sensory and motor ciliopathies may not reflect what really occurs. Copyright © 2014 Elsevier Inc. All rights reserved.

NONINVASIVE METHODS ASSESSMENT BLOOD FLOW IN ANTERIOR SEGMENT AND CLINICAL APPLICATION PERSPECTIVE

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T. N. Kiseleva

2017-01-01

Full Text Available The literature review contains information on the anatomical and physiological features of the vessels of the conjunctiva, iris, ciliary body. There are data on the development and application of new non-invasive methods for the study of hemodynamics in the microvessels of anterior eye segment. To study the blood flow of the anterior segment of the eye, biomycroscopy, photography and videobiomicroscopy, television biomicroscopy of vessels, darkfield visualization, application fluorescence angiography, photoacoustic angiography, orthogonal polarization spectroscopy, laser Doppler flowmetry and OCT-angiography were used in recent years. These methods allow to determine the qualitative and quantitative characteristics of conjunctiva, iris, ciliary body microcirculation. They are highly informative for assess of various drugs effect on the vascular eye system. Investigation of hemodynamics in the eye microvessels is necessary for a fundamental approach to the study of the pathophysiology of systemic circulatory pathologies (with arterial hypertension, diabetes, etc. and changes in regional blood flow in organ of vision disease. Monitoring of anterior segment microcirculation in clinical practice makes possible to monitor the effectiveness of drug and surgical treatment.

Voltage-gated calcium channels of Paramecium cilia.

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Lodh, Sukanya; Yano, Junji; Valentine, Megan S; Van Houten, Judith L

2016-10-01

Paramecium cells swim by beating their cilia, and make turns by transiently reversing their power stroke. Reversal is caused by Ca 2+ entering the cilium through voltage-gated Ca 2+ (Ca V ) channels that are found exclusively in the cilia. As ciliary Ca 2+ levels return to normal, the cell pivots and swims forward in a new direction. Thus, the activation of the Ca V channels causes cells to make a turn in their swimming paths. For 45 years, the physiological characteristics of the Paramecium ciliary Ca V channels have been known, but the proteins were not identified until recently, when the P. tetraurelia ciliary membrane proteome was determined. Three Ca V α1 subunits that were identified among the proteins were cloned and confirmed to be expressed in the cilia. We demonstrate using RNA interference that these channels function as the ciliary Ca V channels that are responsible for the reversal of ciliary beating. Furthermore, we show that Pawn (pw) mutants of Paramecium that cannot swim backward for lack of Ca V channel activity do not express any of the three Ca V 1 channels in their ciliary membrane, until they are rescued from the mutant phenotype by expression of the wild-type PW gene. These results reinforce the correlation of the three Ca V channels with backward swimming through ciliary reversal. The PwB protein, found in endoplasmic reticulum fractions, co-immunoprecipitates with the Ca V 1c channel and perhaps functions in trafficking. The PwA protein does not appear to have an interaction with the channel proteins but affects their appearance in the cilia. © 2016. Published by The Company of Biologists Ltd.

Numerical simulation of flow fields and particle trajectories

DEFF Research Database (Denmark)

Mayer, Stefan

2000-01-01

. The time-dependent flow is approximated with a continuous sequence of steady state creeping flow fields, where metachronously beating ciliary bands are modelled by linear combinations of singularity solutions to the Stokes equations. Generally, the computed flow fields can be divided into an unsteady......A model describing the ciliary driven flow and motion of suspended particles in downstream suspension feeders is developed. The quasi-steady Stokes equations for creeping flow are solved numerically in an unbounded fluid domain around cylindrical bodies using a boundary integral formulation...... in the simulated unsteady ciliary driven flow. A fraction of particles appear to follow trajectories, that resemble experimentally observed particle capture events in the downstream feeding system of the polycheate Sabella penicillus, indicating that particles can be captured by ciliary systems without mechanical...

Primary Cilia, Signaling Networks and Cell Migration

DEFF Research Database (Denmark)

Veland, Iben Rønn

Primary cilia are microtubule-based, sensory organelles that emerge from the centrosomal mother centriole to project from the surface of most quiescent cells in the human body. Ciliary entry is a tightly controlled process, involving diffusion barriers and gating complexes that maintain a unique...... this controls directional cell migration as a physiological response. The ciliary pocket is a membrane invagination with elevated activity of clathrin-dependent endocytosis (CDE). In paper I, we show that the primary cilium regulates TGF-β signaling and the ciliary pocket is a compartment for CDE...... on formation of the primary cilium and CDE at the pocket region. The ciliary protein Inversin functions as a molecular switch between canonical and non-canonical Wnt signaling. In paper II, we show that Inversin and the primary cilium control Wnt signaling and are required for polarization and cell migration...

The mother centriole plays an instructive role in defining cell geometry.

Directory of Open Access Journals (Sweden)

Jessica L Feldman

2007-06-01

Full Text Available Centriole positioning is a key step in establishment and propagation of cell geometry, but the mechanism of this positioning is unknown. The ability of pre-existing centrioles to induce formation of new centrioles at a defined angle relative to themselves suggests they may have the capacity to transmit spatial information to their daughters. Using three-dimensional computer-aided analysis of cell morphology in Chlamydomonas, we identify six genes required for centriole positioning relative to overall cell polarity, four of which have known sequences. We show that the distal portion of the centriole is critical for positioning, and that the centriole positions the nucleus rather than vice versa. We obtain evidence that the daughter centriole is unable to respond to normal positioning cues and relies on the mother for positional information. Our results represent a clear example of "cytotaxis" as defined by Sonneborn, and suggest that centrioles can play a key function in propagation of cellular geometry from one generation to the next. The genes documented here that are required for proper centriole positioning may represent a new class of ciliary disease genes, defects in which would be expected to cause disorganized ciliary position and impaired function.

Mucociliary transport and upper airway disease

International Nuclear Information System (INIS)

Takeuchi, Kazuhiko

2010-01-01

Mucociliary transport so critical in nasal, paranasal sinus, and middle ear physiology is impaired in chronic sinsusitis and otitis media by factors such as increased mucus viscoelasticity, decreased ciliary area, and primary or secondary ciliary immotility. We reviewed the pathophysiology of primary ciliary dyskinesia, otitis media with effusion, chronic sinusitis, and allergic rhinitis in terms of mucociliary transport. Subjects with primary ciliary dyskinesia may experience recurrent middle ear infection, chronic airway infection, predominantly lower-lobe bronchiectasis, male sterility, or situs inversus. Primary ciliary dyskinesia is sometimes difficult to diagnose in cases without situs inversus. Nasal nitric oxide concentration in such patients decreases, although why is unclear. Mutations may involve dynein arm intermediate chain 1 (DNAI1) or dynein arm heavy chain 5 (DNAH5). Mucociliary clearance decreases more in those with otitis media with effusion than in those without, due in part to increased middle ear effusion viscosity. Prognosis is poor in subjects with viscous effusion, which is difficult to clear from the middle ear via the mucociliary system. An understanding of anatomic paranasal sinus variations is thus extremely important in chronic sinusitis when endoscopic sinus surgery is attempted, although recent advances in computed tomography (CT) have enabled paranasal sinus drainage pathways to be delineated more clearly than ever before. (author)

Discinesia ciliar primária: considerações sobre seis casos da síndrome de Kartagener Primary ciliary dyskinesia: considerations regarding six cases of Kartagener syndrome

Directory of Open Access Journals (Sweden)

Hugo Alejandro Vega Ortega

2007-10-01

Full Text Available A discinesia ciliar primária (DCP, anteriormente conhecida como síndrome dos cílios imóveis, é uma doença hereditária autossômica recessiva que inclui vários padrões de defeitos em sua ultra-estrutura ciliar. Sua forma clínica mais grave é a síndrome de Kartagener (SK, a qual é encontrada em 50% dos casos de DCP. A DCP causa deficiência ou mesmo estase no transporte de secreções em todo o trato respiratório, favorecendo a proliferação de vírus e bactérias. Sua incidência varia de 1:20.000 a 1:60.000. Como conseqüência, os pacientes apresentam infecções crônicas e repetidas desde a infância e geralmente são portadores de bronquite, pneumonia, hemoptise, sinusite e infertilidade. As bronquiectasias e outras infecções crônicas podem ser o resultado final das alterações irreversíveis dos brônquios, podendo progredir para cor pulmonale crônico e suas conseqüências. Somente a metade dos pacientes afetados pela DCP apresenta todos os sintomas, condição denominada SK completa; no restante, não ocorre situs inversus, condição denominada SK incompleta. O diagnóstico é feito com base no quadro clínico e confirmado por meio da microscopia eletrônica de transmissão. Como não há tratamento especifico para a DCP, recomenda-se que, tão logo seja feito o diagnóstico, as infecções secundárias sejam tratadas com antibióticos potentes e medidas profiláticas sejam adotadas. Neste trabalho, relatamos seis casos de DCP (cinco casos de SK completa e um caso de SK incompleta e revisamos a literatura sobre o assunto, tendo como foco os aspectos diagnósticos, terapêuticos e clínicos desta doença.Primary ciliary dyskinesia (PCD, previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS, which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1

Primary Ciliary Dyskinesia

Science.gov (United States)

... made to help with CPT, such as: An electric chest clapper, known as a mechanical percussor. An ... your child spends time, including the home and car. Encourage your child to never start smoking. For ...

An organelle-specific protein landscape identifies novel diseases and molecular mechanisms.

Science.gov (United States)

Boldt, Karsten; van Reeuwijk, Jeroen; Lu, Qianhao; Koutroumpas, Konstantinos; Nguyen, Thanh-Minh T; Texier, Yves; van Beersum, Sylvia E C; Horn, Nicola; Willer, Jason R; Mans, Dorus A; Dougherty, Gerard; Lamers, Ideke J C; Coene, Karlien L M; Arts, Heleen H; Betts, Matthew J; Beyer, Tina; Bolat, Emine; Gloeckner, Christian Johannes; Haidari, Khatera; Hetterschijt, Lisette; Iaconis, Daniela; Jenkins, Dagan; Klose, Franziska; Knapp, Barbara; Latour, Brooke; Letteboer, Stef J F; Marcelis, Carlo L; Mitic, Dragana; Morleo, Manuela; Oud, Machteld M; Riemersma, Moniek; Rix, Susan; Terhal, Paulien A; Toedt, Grischa; van Dam, Teunis J P; de Vrieze, Erik; Wissinger, Yasmin; Wu, Ka Man; Apic, Gordana; Beales, Philip L; Blacque, Oliver E; Gibson, Toby J; Huynen, Martijn A; Katsanis, Nicholas; Kremer, Hannie; Omran, Heymut; van Wijk, Erwin; Wolfrum, Uwe; Kepes, François; Davis, Erica E; Franco, Brunella; Giles, Rachel H; Ueffing, Marius; Russell, Robert B; Roepman, Ronald

2016-05-13

Cellular organelles provide opportunities to relate biological mechanisms to disease. Here we use affinity proteomics, genetics and cell biology to interrogate cilia: poorly understood organelles, where defects cause genetic diseases. Two hundred and seventeen tagged human ciliary proteins create a final landscape of 1,319 proteins, 4,905 interactions and 52 complexes. Reverse tagging, repetition of purifications and statistical analyses, produce a high-resolution network that reveals organelle-specific interactions and complexes not apparent in larger studies, and links vesicle transport, the cytoskeleton, signalling and ubiquitination to ciliary signalling and proteostasis. We observe sub-complexes in exocyst and intraflagellar transport complexes, which we validate biochemically, and by probing structurally predicted, disruptive, genetic variants from ciliary disease patients. The landscape suggests other genetic diseases could be ciliary including 3M syndrome. We show that 3M genes are involved in ciliogenesis, and that patient fibroblasts lack cilia. Overall, this organelle-specific targeting strategy shows considerable promise for Systems Medicine.

The response of the ciliated epithelium during and after exposure to ionizing radiation

International Nuclear Information System (INIS)

Baldetorp, B.

1984-10-01

Irradiation of the ciliated tissues of the body gives undesirable sideefects. In vitro irradiation (10 Gy) of the rabbit's trachea shows that 1.5 Gy of indirectly ionizing radiation (50 kV and 6 MV X-ray 60 Co-gamma 1.25 MeV) causes a 20 per cent increase of the ciliary beat frequency lasting 5-10 seconds, followed by a decline to normal ciliary activity during the ensuing course of irradiation. Electron radiation (4 MeV) proved to be three times more effective than photon radiation in regard to the physiological response of the cilia to ionizing radiation. This finding led to introduction of the concept Relative Physiological Efficiency (RPE) in this study, complementing the Relative Biological Efficiency (RBE) concept. This momentary increase in frequency can be caused by a radiation-induced increased hydrolysis of the ATP available in the cilia. The ciliary activity was 20 per cent lower than normal at 45 min following irradiation (10 Gy, 50 kV X-ray), whereupon it increased to 12 per cent above normal activity at two hours after initial irradiation. At re-irradiation (10 Gy, 50 kV X-ray) administered two hours after initial irradiation, the cilia showed a constant rate of activity. In vivo irradiation (10 Gy, 160 kV X-ray) of the trachea of the rabbit caused a heightened activity (10%) during the first three days after irradiation, indicating a stimulation of the ATP-synthesis. During days 4 to 8 after irradiation, the ciliary epithelium's morphology was damaged resulting in reduced transport ability. Repair took place during days 9 and 10 after irradiation, i.e. the function of the ciliary epithelium appeared to be restored. The membrane potential of the ciliary cell, registered during irradiation (10 Gy, 50 kV X-ray) showed no changes, which supports the assumption that the increased ciliary beat frequency recorded during irradiation can be due to rapid radiation-induced biochemical changes that are connected to the motility of the cilia. (author)

SRS-A leukotrienes decrease the activity of human respiratory cilia

DEFF Research Database (Denmark)

Bisgaard, H; Pedersen, M

1987-01-01

We have studied the effects of the slow reacting substance of anaphylaxis (SRS-A) constituents leukotrienes (LT) C4 and D4 on the ciliary activity of human respiratory cells. The ciliary beat frequency on human nasal cells harvested by cell scraping from the inferior turbinate was measured...

Evolution and Structure of Neuromuscular Systems in Spiralian Meiofauna

DEFF Research Database (Denmark)

Bekkouche, Nicolas Tarik

three taxa together forming Gnathifera). Furthermore, we could infer possible plesiomorphic states of Gnathifera such as the paired ventro-lateral nerve chords (shared with many Spiralia) as well as recover putative Gnathifera apomorphies such as the pharyngeal ganglion; all adding new information...... such as the ciliary pattern or a system of pharyngeal canals of possible importance for future comparative approaches. These different studies show that information on rare and phylogenetically isolated animals with their unique combination of neural and muscular characters are necessary to understand the evolution...

A case of Kartagener syndrome with rhinolalia clausa | Raoufi | Pan ...

African Journals Online (AJOL)

Kartagener syndrome is an autosomal recessive genetic ciliary disorder comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. It's the one of primary ciliary dyskinesia disorders with manifestations present from childhood. Most patients of PCD have situs inversus. We present a case of 18 year-old women ...

Histoanatomical study on the uveal coat of eye in mature ostrich

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mohammadali Ebrahimi saadatlou

2015-05-01

Full Text Available In the present study, the uveal coats of 20 healthy adult ostriches were studied anatomically and histologically. At first, the appearance, dimension, structure and vicinity of choroid, ciliary body and iris were evaluated macroscopically. Then they were studied microscopically after preparing histological slides and staining by H&E, Verhoeff, Van Gieson, and P.A.S. Tapetum lucidum was not seen in the choroid. The average thickness of ciliary body was measured as 1.48±0.01 centimeters. Moreover, the number of macroscopic ciliary body process in the ostrich eye was about 120. Iris thickness in the normal state is 0.7 centimeters and the diameter of pupil was measured as 1.2 centimeters. Pupil is round shaped in ostrich. There is a hyaline cartilage membrane between the sclera and choroid. There is bruch's membrane in the choroid and the total thickness of the choroid was measured as 350 µm. The ciliary body was supported by a hyaline cartilage. Skeletal muscle fibers in the ciliary body were seen as separated masses. Epithelium is lacking on the anterior surface of the iris. Iridial muscle fibers were smooth. The posterior epithelium of the iris had two pigmented layers with the inner layer acting as myoepithelial cells. In conclusion, the uveal coat of ostrich was similar to other birds although there were little differences in anatomical dimensions and histological characteristics

A feed-forward regulation of endothelin receptors by c-Jun in human non-pigmented ciliary epithelial cells and retinal ganglion cells.

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Junming Wang

Full Text Available c-Jun, c-Jun N-terminal kinase(JNK and endothelin B (ETB receptor have been shown to contribute to the pathogenesis of glaucoma. Previously, we reported that an increase of c-Jun and CCAAT/enhancer binding protein β (C/EBPβ immunohistostaining is associated with upregulation of the ETB receptor within the ganglion cell layer of rats with elevated intraocular pressure (IOP. In addition, both transcription factors regulate the expression of the ETB receptor in human non-pigmented ciliary epithelial cells (HNPE. The current study addressed the mechanisms by which ET-1 produced upregulation of ET receptors in primary rat retinal ganglion cells (RGCs and HNPE cells. Treatment of ET-1 and ET-3 increased the immunocytochemical staining of c-Jun and C/EBPβ in primary rat RGCs and co-localization of both transcription factors was observed. A marked increase in DNA binding activity of AP-1 and C/EBPβ as well as elevated protein levels of c-Jun and c-Jun-N-terminal kinase (JNK were detected following ET-1 treatment in HNPE cells. Overexpression of ETA or ETB receptor promoted the upregulation of c-Jun and also elevated its promoter activity. In addition, upregulation of C/EBPβ augmented DNA binding and mRNA expression of c-Jun, and furthermore, the interaction of c-Jun and C/EBPβ was confirmed using co-immunoprecipitation. Apoptosis of HNPE cells was identified following ET-1 treatment, and overexpression of the ETA or ETB receptor produced enhanced apoptosis. ET-1 mediated upregulation of c-Jun and C/EBPβ and their interaction may represent a novel mechanism contributing to the regulation of endothelin receptor expression. Reciprocally, c-Jun was also found to regulate the ET receptors and C/EBPβ appeared to play a regulatory role in promoting expression of c-Jun. Taken together, the data suggests that ET-1 triggers the upregulation of c-Jun through both ETA and ETB receptors, and conversely c-Jun also upregulates endothelin receptor expression

Ciliary neurotrophic factor inhibits brain and peripheral tumor necrosis factor production and, when coadministered with its soluble receptor, protects mice from lipopolysaccharide toxicity.

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Benigni, F; Villa, P; Demitri, M T; Sacco, S; Sipe, J D; Lagunowich, L; Panayotatos, N; Ghezzi, P

1995-07-01

The receptor of ciliary neurotrophic factor (CNTF) contains the signal transduction protein gp130, which is also a component of the receptors of cytokines such as interleukin (IL)-6, leukemia-inhibitory factor (LIF), IL-11, and oncostatin M. This suggests that these cytokines might share common signaling pathways. We previously reported that CNTF augments the levels of corticosterone (CS) and of IL-6 induced by IL-1 and induces the production of the acute-phase protein serum amyloid A (SAA). Since the elevation of serum CS is an important feedback mechanism to limit the synthesis of proinflammatory cytokines, particularly tumor necrosis factor (TNF), we have investigated the effect of CNTF on both TNF production and lipopolysaccharide (LPS) toxicity. To induce serum TNF levels, LPS was administered to mice at 30 mg/kg i.p. and CNTF was administered as a single dose of 10 micrograms/mouse i.v., either alone or in combination with its soluble receptor sCNTFR alpha at 20 micrograms/mouse. Serum TNF levels were the measured by cytotoxicity on L929 cells. In order to measure the effects of CNTF on LPS-induced TNF production in the brain, mice were injected intracerebroventricularly (i.c.v.) with 2.5 micrograms/kg LPS. Mouse spleen cells cultured for 4 hr with 1 microgram LPS/ml, with or without 10 micrograms CNTF/ml, were also analyzed for TNF production. CNTF, administered either alone or in combination with its soluble receptor, inhibited the induction of serum TNF levels by LPS. This inhibition was also observed in the brain when CNTF and LPS were administered centrally. In vitro, CNTF only marginally affected TNF production by LPS-stimulated mouse splenocytes, but it acted synergistically with dexamethasone (DEX) in inhibiting TNF production. Most importantly, CNTF administered together with sCNTFR alpha protected mice against LPS-induced mortality. These data suggest that CNTF might act as a protective cytokine against TNF-mediated pathologies both in the brain and

Ciliary derived neurotrophic factor protects oligodendrocytes against radiation induced damage in vitro by a mechanism independent of a proliferative effect

International Nuclear Information System (INIS)

Evans, Andrew J.; Mabie, Peter C.; Kessler, Jack A.; Vikram, Bhadrasain

1997-01-01

Purpose/Objective: Radiation-induced damage to the central nervous system in the from of myelopathy is a dose-limiting complication in the treatment of tumors situated in or close to the spinal cord. The target cell for this damage is not definitively identified, but demyelination due to oligodendrocyte damage is strongly implicated. Multiple neurotrophic factors have recently been identified which demonstrate a survival effect on oligodendrocytes. We investigated the effect of Ciliary Derived Neurotrophic Factor (CNTF), Neurotrophin-3 (NT-3) and Nerve Growth Factor (NGF) on the radiosensitivity of oligodendrocytes in vitro to determine if this may ameliorate radiation damage, as a model for reducing myelopathy in vivo. Materials and Methods: Mature oligodendrocytes were cultured from the cortex of newborn Sprague-Dawley white rats and maintained on poly-d-lysine plates. The experimental arm was exposed to CNTF (0.01-100ng/ml), NGF (100ng/ml) or NT-3 (20ng/ml) for 24 hours prior to radiation, and control and experimental arms radiated using a cobalt 60 irradiator at a dose rate of .87 Gy/min with doses from 2 Gy to 10 Gy. Oligodendrocytes were identified using an O4 antibody, assessed for viability at 5 days using an MTT assay and counted using a phase contrast microscope. Combination studies of CNTF and NT-3 were also performed. BrdU studies were performed to determine if the various neurotrophins induced proliferation, with BrdU added for the 24 hour period prior to radiation only, for the 5 day period following radiation only, or for both periods combined. Results: The proportion of mature oligodendrocytes surviving 5 days after irradiation was not significantly increased by NGF, and was only modestly increased by NT-3. However, CNTF significantly increased the surviving proportion at all doses The addition of NT-3 to CNTF did not further increase the proportion of oligodendrocytes surviving. CNTF dose escalation studies confirmed 20ng/ml as an optimal dose. Brd

Neovascular glaucoma in a child: an unusual presentation of medulloepithelioma

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Kassa, Enoch

2014-04-01

Full Text Available [english] A healthy 12 month old infant without significant medical history presented with left eye redness for one week. Ophthalmic examination showed elevated intraocular pressure with iris neovascularization in the affected eye with increased optic nerve cupping. Scleral depression revealed a ciliary body mass in the supratemporal quadrant. A large, non-pigmented, vascular mass was noted; biopsy results showed multilayered cords, tubules, and sheets resembling primitive medullary epithelium arising from the ciliary body. The patient was diagnosed with medulloepithelioma. The patient underwent enucleation of the affected eye. Medulloepithelioma is a rare but important cause of neovascular glaucoma in the pediatric population. This case will focus on the characteristics of medulloepthelioma and the differential diagnosis for a non-pigmented ciliary body mass in a child.

Lens stem cells may reside outside the lens capsule: an hypothesis

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Meyer Rita A

2007-06-01

Full Text Available Abstract In this paper, we consider the ocular lens in the context of contemporary developments in biological ideas. We attempt to reconcile lens biology with stem cell concepts and a dearth of lens tumors. Historically, the lens has been viewed as a closed system, in which cells at the periphery of the lens epithelium differentiate into fiber cells. Theoretical considerations led us to question whether the intracapsular lens is indeed self-contained. Since stem cells generate tumors and the lens does not naturally develop tumors, we reasoned that lens stem cells may not be present within the capsule. We hypothesize that lens stem cells reside outside the lens capsule, in the nearby ciliary body. Our ideas challenge the existing lens biology paradigm. We begin our discussion with lens background information, in order to describe our lens stem cell hypothesis in the context of published data. Then we present the ciliary body as a possible source for lens stem cells, and conclude by comparing the ocular lens with the corneal epithelium.

Qualitative Assessment of Ultrasound Biomicroscopic Images Using Standard Photographs: The Liwan Eye Study

Science.gov (United States)

Jiang, Yuzhen; Huang, Wenyong; Huang, Qunxiao; Zhang, Jian; Foster, Paul J.

2010-01-01

Objective. To classify anatomic features related to anterior chamber angles by a qualitative assessment system based on ultrasound biomicroscopy (UBM) images. Methods. Cases of primary angle-closure suspect (PACS), defined by pigmented trabecular meshwork that is not visible in two or more quadrants on static gonioscopy (cases) and systematically selected subjects (1 of every 10) who did not meet this criterion (controls) were enrolled during a population-based survey in Guangzhou, China. All subjects underwent UBM examination. A set of standard UBM images was used to qualitatively classify anatomic features related to the angle configuration, including iris thickness, iris convexity, iris angulation, ciliary body size, and ciliary process position. All analysis was conducted on right eye images. Results. Based on the qualitative grades, the difference in overall iris thickness between gonioscopically narrow eyes (n = 117) and control eyes (n = 57) was not statistically significant. The peripheral one third of the iris tended to be thicker in all quadrants of the PACS eyes, although the difference was statistically significant only in the superior quadrant (P = 0.008). No significant differences were found in the qualitative classifications of iris insertion, iris angulation, ciliary body size, and ciliary process position. The findings were similar when compared with the control group of eyes with wide angles in all quadrants. Conclusions. Basal iris thickness seems to be more relevant to narrow angle configuration than to overall iris thickness. Otherwise, the anterior rotation and size of the ciliary body, the iris insertion, and the overall iris thickness are comparable in narrow- and wide-angle eyes. PMID:19834039

Ciliopathies: Genetics in Pediatric Medicine.

Science.gov (United States)

Oud, Machteld M; Lamers, Ideke J C; Arts, Heleen H

2017-03-01

Ciliary disorders , which are also referred to as ciliopathies , are a group of hereditary disorders that result from dysfunctional cilia. The latter are cellular organelles that stick up from the apical plasma membrane. Cilia have important roles in signal transduction and facilitate communications between cells and their surroundings. Ciliary disruption can result in a wide variety of clinically and genetically heterogeneous disorders with overlapping phenotypes. Because cilia occur widespread in our bodies many organs and sensory systems can be affected when they are dysfunctional. Ciliary disorders may be isolated or syndromic, and common features are cystic liver and/or kidney disease, blindness, neural tube defects, brain anomalies and intellectual disability, skeletal abnormalities ranging from polydactyly to abnormally short ribs and limbs, ectodermal defects, obesity, situs inversus , infertility, and recurrent respiratory tract infections. In this review, we summarize the features, frequency, morbidity, and mortality of each of the different ciliopathies that occur in pediatrics. The importance of genetics and the occurrence of genotype-phenotype correlations are indicated, and advances in gene identification are discussed. The use of next-generation sequencing by which a gene panel or all genes can be screened in a single experiment is highlighted as this technology significantly lowered costs and time of the mutation detection process in the past. We discuss the challenges of this new technology and briefly touch upon the use of whole-exome sequencing as a diagnostic test for ciliary disorders. Finally, a perspective on the future of genetics in the context of ciliary disorders is provided.

Digitaria ciliaris (Retz.) Koeler

Czech Academy of Sciences Publication Activity Database

Danihelka, Jiří

2011-01-01

Roč. 46, č. 1 (2011), s. 85-87 ISSN 1211-5258 R&D Projects: GA MŠk LC06073 Institutional research plan: CEZ:AV0Z60050516 Keywords : alien species * Czech Republic * neophyte Subject RIV: EF - Botanics

In Silico and Fluorescence In Situ Hybridization Mapping Reveals Collinearity between the Pennisetum squamulatum Apomixis Carrier-Chromosome and Chromosome 2 of Sorghum and Foxtail Millet

OpenAIRE

Sapkota, Sirjan; Conner, Joann A.; Hanna, Wayne W.; Simon, Bindu; Fengler, Kevin; Deschamps, St?phane; Cigan, Mark; Ozias-Akins, Peggy

2016-01-01

Apomixis, or clonal propagation through seed, is a trait identified within multiple species of the grass family (Poaceae). The genetic locus controlling apomixis in Pennisetum squamulatum (syn Cenchrus squamulatus) and Cenchrus ciliaris (syn Pennisetum ciliare, buffelgrass) is the apospory-specific genomic region (ASGR). Previously, the ASGR was shown to be highly conserved but inverted in marker order between P. squamulatum and C. ciliaris based on fluorescence in situ hybridization (FISH) a...

Novel Bioconjugation Strategy Using Elevated Hydrostatic Pressure: A Case Study for the Site-Specific Attachment of Polyethylene Glycol (PEGylation) of Recombinant Human Ciliary Neurotrophic Factor.

Science.gov (United States)

Wang, Qi; Zhang, Chun; Guo, Fangxia; Li, Zenglan; Liu, Yongdong; Su, Zhiguo

2017-11-15

In this paper, we reported a novel strategy for the site-specific attachment of polyethylene glycol (PEGylation) of proteins using elevated hydrostatic pressure. The process was similar to the conventional one except the reactor was under elevated hydrostatic pressure. The model protein was recombinant human ciliary neurotrophic factor (rhCNTF), and the reagent was monomethoxy-polyethylene glycol-maleimide (mPEG-MAL). PEGylation with mPEG (40 kDa)-MAL at pH 7.0 under normal pressure for 5 h achieved a less than 5% yield. In comparison, when the pressure was elevated, the PEGylation yield was increased dramatically, reaching nearly 90% at 250 MPa. Furthermore, the following phenomena were observed: (1) high-hydrostatic-pressure PEGylation (HHPP) could operate at a low reactant ratio of 1:1.2 (rhCNTF to mPEG-MAL), while the conventional process needs a much-higher ratio. (2) Short and long chains of PEG gave a similar yield of 90% in HHPP, while the conventional yield for the short chain of the PEG was higher than that of the long chain. (3) The reaction pH in the range of 7.0 to 8.0 had almost no influence upon the yield of HHPP, while the PEGylation yield was significantly increased by a factor of three from pH 7.0 to 8.0 at normal pressure. Surface accessibility analysis was performed using GRASP2 software, and we found that Cys17 of rhCNTF was located at the concave patches, which may have steric hindrance for the PEG to approach. The speculated benefit of HHPP was the facilitation of target-site exposure, reducing the steric hindrance and making the reaction much easier. Structure and activity analysis demonstrated that the HHPP product was comparable to the PEGylated rhCNTF prepared through a conventional method. Overall, this work demonstrated that HHPP, as we proposed, may have application potentials in various conjugations of biomacromolecules.

Analysis of eye health in the Thai population.

Science.gov (United States)

Yospaiboon, Yosanan; Yospaiboon, Kasem; Ratanapakorn, Tanapat; Sinawat, Suthasinee; Sanguansak, Thuss; Bhoomibunchoo, Chavakij

2012-07-01

To assess national eye health as a baseline for improving medical education, services and research. Information on eye illness among out- and in-patients was based on hospital claims from the three national health insurance schemes in 2010. The data were analyzed by age groups. In 2010, 4,982,025 out-patient visits and 187,080 in-patient admissions were reported. The five most common outpatient eye conditions were disorders of the (1) conjunctiva (H10-H13) (2) eyelid, lacrimal system and orbit (H00-H06) (3) lens (H25-28) (4) sclera, cornea, iris and ciliary body (H15-H22) and (5) glaucoma (H40-H42). The five most common inpatient eye diseases were (1) disorders of lens (H25-H28) (2) conjunctiva (H10-H13) (3) sclera, cornea, iris and ciliary body (H15-H22) (4) choroid and retina (H30-H35) and (5) glaucoma (H40-H42). Information on the most frequent eye diseases-including the types and frequency of high risk conditions, the diseases causing the most impact on vision and resulting in the highest health expenditures-will be used to (a) revise the curricula for medical education (b) to improve eye care services and (c) to improve access to data for research themes tailored to local and national needs.

[Microscopic structure of the epithelium of the oviducts in cows during the estrus cycle].

Science.gov (United States)

Uhrín, V

1983-03-01

The mucous membrane of a cow is covered with ciliary and secretory cells. The so-called basal cells occur at the basal membrane. The counts of ciliary cells vary during the sexual cycle: they reach the maximum (up to 68%) during oestrus. About 13% of cells lose cilia during metoestrus and at the beginning of dioestrus. Reciliation occurs during pro-oestrus. Light and dark ciliary cells can be discerned by the staining of cytoplasm and by the density of nuclei. A higher variability was found in the secretory cells. There are light and dark cells, cells with a wedge shape and rod-shaped cells. Their frequency and function are discussed. Mitoses of epithelium were found in rare cases. The relative volume of epithelium and the mucous membrane of connective tissues change during the sexual cycle. The volume of secretory cells increases during metoestrus and dioestrus and the volume of ciliary cells increases during pro-oestrus and heat. The volume of nuclei decreases in metoestrus and mainly in dioestrus. PAS positive granules occur in the cytoplasm of secretory cells, mainly during metoestrus, in the apical regions. Ptyalin-resistant polysaccharides, besides glycogen, were detected in the cells. The occurrence rate of lipids varies just slightly during the oestrous cycle.

The Golgin GMAP210/TRIP11 anchors IFT20 to the Golgi complex.

Directory of Open Access Journals (Sweden)

John A Follit

2008-12-01

Full Text Available Eukaryotic cells often use proteins localized to the ciliary membrane to monitor the extracellular environment. The mechanism by which proteins are sorted, specifically to this subdomain of the plasma membrane, is almost completely unknown. Previously, we showed that the IFT20 subunit of the intraflagellar transport particle is localized to the Golgi complex, in addition to the cilium and centrosome, and hypothesized that the Golgi pool of IFT20 plays a role in sorting proteins to the ciliary membrane. Here, we show that IFT20 is anchored to the Golgi complex by the golgin protein GMAP210/Trip11. Mice lacking GMAP210 die at birth with a pleiotropic phenotype that includes growth restriction, ventricular septal defects of the heart, omphalocele, and lung hypoplasia. Cells lacking GMAP210 have normal Golgi structure, but IFT20 is no longer localized to this organelle. GMAP210 is not absolutely required for ciliary assembly, but cilia on GMAP210 mutant cells are shorter than normal and have reduced amounts of the membrane protein polycystin-2 localized to them. This work suggests that GMAP210 and IFT20 function together at the Golgi in the sorting or transport of proteins destined for the ciliary membrane.

Chlamydomonas DYX1C1/PF23 is essential for axonemal assembly and proper morphology of inner dynein arms.

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Ryosuke Yamamoto

2017-09-01

Full Text Available Cytoplasmic assembly of ciliary dyneins, a process known as preassembly, requires numerous non-dynein proteins, but the identities and functions of these proteins are not fully elucidated. Here, we show that the classical Chlamydomonas motility mutant pf23 is defective in the Chlamydomonas homolog of DYX1C1. The pf23 mutant has a 494 bp deletion in the DYX1C1 gene and expresses a shorter DYX1C1 protein in the cytoplasm. Structural analyses, using cryo-ET, reveal that pf23 axonemes lack most of the inner dynein arms. Spectral counting confirms that DYX1C1 is essential for the assembly of the majority of ciliary inner dynein arms (IDA as well as a fraction of the outer dynein arms (ODA. A C-terminal truncation of DYX1C1 shows a reduction in a subset of these ciliary IDAs. Sucrose gradients of cytoplasmic extracts show that preassembled ciliary dyneins are reduced compared to wild-type, which suggests an important role in dynein complex stability. The role of PF23/DYX1C1 remains unknown, but we suggest that DYX1C1 could provide a scaffold for macromolecular assembly.

De novo 14q24.2q24.3 microdeletion including IFT43 is associated with intellectual disability, skeletal anomalies, cardiac anomalies, and myopia.

Science.gov (United States)

Stokman, Marijn F; Oud, Machteld M; van Binsbergen, Ellen; Slaats, Gisela G; Nicolaou, Nayia; Renkema, Kirsten Y; Nijman, Isaac J; Roepman, Ronald; Giles, Rachel H; Arts, Heleen H; Knoers, Nine V A M; van Haelst, Mieke M

2016-06-01

We report an 11-year-old girl with mild intellectual disability, skeletal anomalies, congenital heart defect, myopia, and facial dysmorphisms including an extra incisor, cup-shaped ears, and a preauricular skin tag. Array comparative genomic hybridization analysis identified a de novo 4.5-Mb microdeletion on chromosome 14q24.2q24.3. The deleted region and phenotype partially overlap with previously reported patients. Here, we provide an overview of the literature on 14q24 microdeletions and further delineate the associated phenotype. We performed exome sequencing to examine other causes for the phenotype and queried genes present in the 14q24.2q24.3 microdeletion that are associated with recessive disease for variants in the non-deleted allele. The deleted region contains 65 protein-coding genes, including the ciliary gene IFT43. Although Sanger and exome sequencing did not identify variants in the second IFT43 allele or in other IFT complex A-protein-encoding genes, immunocytochemistry showed increased accumulation of IFT-B proteins at the ciliary tip in patient-derived fibroblasts compared to control cells, demonstrating defective retrograde ciliary transport. This could suggest a ciliary defect in the pathogenesis of this disorder. © 2016 Wiley Periodicals, Inc. © 2016 Wiley Periodicals, Inc.

Novel Insights into the Development and Function of Cilia Using the Advantages of the Paramecium Cell and Its Many Cilia

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Junji Yano

2015-07-01

Full Text Available Paramecium species, especially P. tetraurelia and caudatum, are model organisms for modern research into the form and function of cilia. In this review, we focus on the ciliary ion channels and other transmembrane proteins that control the beat frequency and wave form of the cilium by controlling the signaling within the cilium. We put these discussions in the context of the advantages that Paramecium brings to the understanding of ciliary motility: mutants for genetic dissections of swimming behavior, electrophysiology, structural analysis, abundant cilia for biochemistry and modern proteomics, genomics and molecular biology. We review the connection between behavior and physiology, which allows the cells to broadcast the function of their ciliary channels in real time. We build a case for the important insights and advantages that this model organism continues to bring to the study of cilia.

Novel Insights into the Development and Function of Cilia Using the Advantages of the Paramecium Cell and Its Many Cilia

Science.gov (United States)

Yano, Junji; Valentine, Megan S.; Van Houten, Judith L.

2015-01-01

Paramecium species, especially P. tetraurelia and caudatum, are model organisms for modern research into the form and function of cilia. In this review, we focus on the ciliary ion channels and other transmembrane proteins that control the beat frequency and wave form of the cilium by controlling the signaling within the cilium. We put these discussions in the context of the advantages that Paramecium brings to the understanding of ciliary motility: mutants for genetic dissections of swimming behavior, electrophysiology, structural analysis, abundant cilia for biochemistry and modern proteomics, genomics and molecular biology. We review the connection between behavior and physiology, which allows the cells to broadcast the function of their ciliary channels in real time. We build a case for the important insights and advantages that this model organism continues to bring to the study of cilia. PMID:26230712

Maintaining protein composition in cilia.

Science.gov (United States)

Stephen, Louise A; Elmaghloob, Yasmin; Ismail, Shehab

2017-12-20

The primary cilium is a sensory organelle that is vital in regulating several signalling pathways. Unlike most organelles cilia are open to the rest of the cell, not enclosed by membranes. The distinct protein composition is crucial to the function of cilia and many signalling proteins and receptors are specifically concentrated within distinct compartments. To maintain this composition, a mechanism is required to deliver proteins to the cilium whilst another must counter the entropic tendency of proteins to distribute throughout the cell. The combination of the two mechanisms should result in the concentration of ciliary proteins to the cilium. In this review we will look at different cellular mechanisms that play a role in maintaining the distinct composition of cilia, including regulation of ciliary access and trafficking of ciliary proteins to, from and within the cilium.

Revealing the Molecular Structure and the Transport Mechanism at the Base of Primary Cilia Using Superresolution STED Microscopy

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Yang, Tung-Lin

The primary cilium is an organelle that serves as a signaling center of the cell and is involved in the hedgehog signaling, cAMP pathway, Wnt pathways, etc. Ciliary function relies on the transportation of molecules between the primary cilium and the cell, which is facilitated by intraflagellar transport (IFT). IFT88, one of the important IFT proteins in complex B, is known to play a role in the formation and maintenance of cilia in various types of organisms. The ciliary transition zone (TZ), which is part of the gating apparatus at the ciliary base, is home to a large number of ciliopathy molecules. Recent studies have identified important regulating elements for TZ gating in cilia. However, the architecture of the TZ region and its arrangement relative to intraflagellar transport (IFT) proteins remain largely unknown, hindering the mechanistic understanding of the regulation processes. One of the major challenges comes from the tiny volume at the ciliary base packed with numerous proteins, with the diameter of the TZ close to the diffraction limit of conventional microscopes. Using a series of stimulated emission depletion (STED) superresolution images mapped to electron microscopy images, we analyzed the structural organization of the ciliary base. Subdiffraction imaging of TZ components defines novel geometric distributions of RPGRIP1L, MKS1, CEP290, TCTN2 and TMEM67, shedding light on their roles in TZ structure, assembly, and function. We found TCTN2 at the outmost periphery of the TZ close to the ciliary membrane, with a 227+/-18 nm diameter. TMEM67 was adjacent to TCTN2, with a 205+/-20 nm diameter. RPGRIP1L was localized toward the axoneme at the same axial level as TCTN2 and TMEM67, with a 165+/-8 nm diameter. MKS1 was situated between TMEM67 and RPGRIP1L, with an 186+/-21 nm diameter. Surprisingly, CEP290 was localized at the proximal side of the TZ close to the distal end of the centrin-labeled basal body. The lateral width was unexpectedly close to

Embryonic and post-embryonic development of the polyclad flatworm Maritigrella crozieri; implications for the evolution of spiralian life history traits

Science.gov (United States)

2010-01-01

Background Planktonic life history stages of spiralians share some muscular, nervous and ciliary system characters in common. The distribution of these characters is patchy and can be interpreted either as the result of convergent evolution, or as the retention of primitive spiralian larval features. To understand the evolution of these characters adequate taxon sampling across the Spiralia is necessary. Polyclad flatworms are the only free-living Platyhelminthes that exhibit a continuum of developmental modes, with direct development at one extreme, and indirect development via a trochophore-like larval stage at the other. Here I present embryological and larval anatomical data from the indirect developing polyclad Maritrigrella crozieri, and consider these data within a comparative spiralian context. Results After 196 h hours of embryonic development, M. crozieri hatches as a swimming, planktotrophic larva. Larval myoanatomy consists of an orthogonal grid of circular and longitudinal body wall muscles plus parenchymal muscles. Diagonal body wall muscles develop over the planktonic period. Larval neuroanatomy consists of an apical plate, neuropile, paired nerve cords, a peri-oral nerve ring, a medial nerve, a ciliary band nerve net and putative ciliary photoreceptors. Apical neural elements develop first followed by posterior perikarya and later pharyngeal neural elements. The ciliated larva is encircled by a continuous, pre-oral band of longer cilia, which follows the distal margins of the lobes; it also possesses distinct apical and caudal cilia. Conclusions Within polyclads heterochronic shifts in the development of diagonal bodywall and pharyngeal muscles are correlated with life history strategies and feeding requirements. In contrast to many spiralians, M. crozieri hatch with well developed nervous and muscular systems. Comparisons of the ciliary bands and apical organs amongst spiralian planktonic life-stages reveal differences; M. crozieri lack a distinct

EVALUATION OF IRIDOCILIARY AND LENTICULAR ELASTICITY USING SHEAR-WAVE ELASTOGRAPHY IN RABBIT EYES

OpenAIRE

Efstathios T. Detorakis; Eleni E. Drakonaki; Harilaos Ginis; Nikolaos Karyotakis; Ioannis G. Pallikaris

2014-01-01

Introduction: A previous study has employed shear-wave ultrasound elastographic imaging to assess corneal rigidity in an ex-vivo porcine eye model. This study employs the same modality in vivo in a rabbit eye model in order to assess lens, ciliary body and total ocular rigidity changes following the instillation of atropine and pilocarpine. Methods: Ten non-pigmented female rabbits were examined. Measurements of the lens, ciliary body and total ocular rigidity as well as lens thickness and an...

Morphological evaluation of buffelgrass cultivar “Lucero INTA-PEMAN” in drought conditions

OpenAIRE

GRIFFA, S.; RIBOTTA, A; LUNA, C; BOLLATI, G; LÓPEZ COLOMBA, E.; TOMMASINO, E; CARLONI, E; QUIROGA, M.; GRUNBERG, K

2011-01-01

In searching for new cultivars that are better adapted to edapho-climatic constraints existing in northwestern Argentina, mainly drought and salinity stress, a hybrid of buffelgrass (Cenchrus ciliaris L.) named Lucero INTA PEMAN was obtained by controlled crosses at the Instituto de Fitopatología y Fisiología Vegetal, INTA. The objective was to morphologically evaluate and compare Cenchrus ciliaris cv Lucero with Texas-4464, Biloela and Molopo cultivars in Dean Funes (North of the Province of...

Uncovering the Forgotten Effect of Superior Cervical Ganglia on Pupil Diameter in Subarachnoid Hemorrhage: An Experimental Study.

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Onen, Mehmet Resid; Yilmaz, Ilhan; Ramazanoglu, Leyla; Aydin, Mehmet Dumlu; Keles, Sadullah; Baykal, Orhan; Aydin, Nazan; Gundogdu, Cemal

2018-01-01

To investigate the relationship between neuron density of the superior cervical sympathetic ganglia and pupil diameter in subarachnoid hemorrhage. This study was conducted on 22 rabbits; 5 for the baseline control group, 5 for the SHAM group and 12 for the study group. Pupil diameters were measured via sunlight and ocular tomography on day 1 as the control values. Pupil diameters were re-measured after injecting 0.5 cc saline to the SHAM group, and autologous arterial blood into the cisterna magna of the study group. After 3 weeks, the brain, superior cervical sympathetic ganglia and ciliary ganglia were extracted with peripheral tissues bilaterally and examined histopathologically. Pupil diameters were compared with neuron densities of the sympathetic ganglia and ciliary ganglia which were examined using stereological methods. Baseline values were; normal pupil diameter 7.180±620 ?m and mean neuron density of the superior cervical sympathetic ganglia 6.321±510/mm3, degenerated neuron density of ciliary ganglia was 5±2/mm3 after histopathological examination in the control group. These values were measured as 6.850±578 ?m, 5.950±340/mm3 and 123±39/mm3 in the SHAM group and 9.910±840 ?m, 7.950±764/mm3 and 650±98/mm3 in the study group. A linear relationship was determined between neuron density of the superior cervical sympathetic ganglia and pupil diameters (p < 0.005). Degenerated ciliary ganglia neuron density had an inverse effect on pupil diameters in all groups (p < 0.0001). Highly degenerated neuron density of the ciliary ganglion is not responsible for pupil dilatation owing to parasympathetic pupilloconstrictor palsy, but high neuron density of the pupillodilatatory superior cervical sympathetic ganglia should be considered an important factor for pupil dilatation.

Effects of a radiosensitizer and radiation on the ciliated mucous membrane

International Nuclear Information System (INIS)

Albertsson, M.

1985-01-01

Fractionated irradiation (2 Gy/F, TD:2-20 Gy) in vivo on the ciliated epithelium of the rabbit's trachea, caused measurable physiological alternations ten consecutive days after completion of irradiation with an initial heightening of the ciliary activity after 2 and 4 Gy, followed by a dose-dependent decrease. On scanning electron microscopy-pictures, knobs were observed on the cilia with the number being related to the dose. The addition of misonidazole potentiated the effects described above, with an enhancement ratio ( physiologically) of 1.2 and an enhancement ratio (morphologically) of 1.6. Furthermore, administration of misonidazole to the rabbits caused an increased vascularity in the subepithelial layer of the trachea, directly correlated to an oedema in the same region. Single doses (2,2.5,5,10,15,20,25, and 30 Gy) were given to the trachea in vivo and daily investigation of the ciliary beating and morphologic examinations of the tissue were made for ten days. The ciliary activity showed a dose-dependent reduction of about 50% after 30 Gy. A development of damage, in relation to the dose, was observed in the cilia. The changes were blebs, swollen tips, bent and curved tips, and broken cilia clustered together. During in vitro irradiation with 10 Gy Single Dose, an increase of the ciliary activity to about 25 % of its original value was observed without any morphological changes, while in vivo irradiation and examinations during 10 days thereafter, showed three different phases, day 1-3: Stimulation phase, day 4-8: Damage-phase, day 9-10: Repair-phase. The ciliary epithelium offers an exceptional system for the study of early radiobiological effects, since mutual comparisons can be made between physiology and morphology. (Author)

IFT46 plays an essential role in cilia development

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Lee, Mi-Sun; Hwang, Kyu-Seok; Oh, Hyun-Woo; Ji-Ae, Kim; Kim, Hyun-Taek; Cho, Hyun-Soo; Lee, Jeong-Ju; Ko, Je Yeong; Choi, Jung-Hwa; Jeong, Yun-Mi; You, Kwan-Hee; Kim, Joon; Park, Doo-Sang; Nam, Ki-Hoan; Aizawa, Shinichi; Kiyonari, Hiroshi; Shioi, Go; Park, Jong-Hoon; Zhou, Weibin; Kim, Nam-Soon; Kim, Cheol-Hee

2015-01-01

Cilia are microtubule-based structures that project into the extracellular space. Ciliary defects are associated with several human diseases, including polycystic kidney disease, primary ciliary dyskinesia, left-right axis patterning, hydrocephalus and retinal degeneration. However, the genetic and cellular biological control of ciliogenesis remains poorly understood. The IFT46 is one of the highly conserved intraflagellar transport complex B proteins. In zebrafish, ift46 is expressed in various ciliated tissues such as Kupffer’s vesicle, pronephric ducts, ears and spinal cord. We show that ift46 is localized to the basal body. Knockdown of ift46 gene results in multiple phenotypes associated with various ciliopathies including kidney cysts, pericardial edema and ventral axis curvature. In ift46 morphants, cilia in kidney and spinal canal are shortened and abnormal. Similar ciliary defects are observed in otic vesicles, lateral line hair cells, olfactory pits, but not in Kupffer’s vesicle. To explore the functions of Ift46 during mouse development, we have generated Ift46 knock-out mice. The Ift46 mutants have developmental defects in brain, neural tube and heart. In particular Ift46(−/−) homozygotes displays randomization of the embryo heart looping, which is a hallmark of defective left-right (L/R) axis patterning. Taken together, our results demonstrated that IFT46 has an essential role in vertebrate ciliary development. PMID:25722189

Self-Sustained Oscillatory Sliding Movement of Doublet Microtubules and Flagellar Bend Formation.

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Sumio Ishijima

Full Text Available It is well established that the basis for flagellar and ciliary movements is ATP-dependent sliding between adjacent doublet microtubules. However, the mechanism for converting microtubule sliding into flagellar and ciliary movements has long remained unresolved. The author has developed new sperm models that use bull spermatozoa divested of their plasma membrane and midpiece mitochondrial sheath by Triton X-100 and dithiothreitol. These models enable the observation of both the oscillatory sliding movement of activated doublet microtubules and flagellar bend formation in the presence of ATP. A long fiber of doublet microtubules extruded by synchronous sliding of the sperm flagella and a short fiber of doublet microtubules extruded by metachronal sliding exhibited spontaneous oscillatory movements and constructed a one beat cycle of flagellar bending by alternately actuating. The small sliding displacement generated by metachronal sliding formed helical bends, whereas the large displacement by synchronous sliding formed planar bends. Therefore, the resultant waveform is a half-funnel shape, which is similar to ciliary movements.

Ciliopathies with Skeletal Anomalies and Renal Insufficiency due to Mutations in the IFT-A Gene WDR19

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Bredrup, Cecilie; Saunier, Sophie; Oud, Machteld M.; Fiskerstrand, Torunn; Hoischen, Alexander; Brackman, Damien; Leh, Sabine M.; Midtbø, Marit; Filhol, Emilie; Bole-Feysot, Christine; Nitschké, Patrick; Gilissen, Christian; Haugen, Olav H.; Sanders, Jan-Stephan F.; Stolte-Dijkstra, Irene; Mans, Dorus A.; Steenbergen, Eric J.; Hamel, Ben C.J.; Matignon, Marie; Pfundt, Rolph; Jeanpierre, Cécile; Boman, Helge; Rødahl, Eyvind; Veltman, Joris A.; Knappskog, Per M.; Knoers, Nine V.A.M.; Roepman, Ronald; Arts, Heleen H.

2011-01-01

A subset of ciliopathies, including Sensenbrenner, Jeune, and short-rib polydactyly syndromes are characterized by skeletal anomalies accompanied by multiorgan defects such as chronic renal failure and retinitis pigmentosa. Through exome sequencing we identified compound heterozygous mutations in WDR19 in a Norwegian family with Sensenbrenner syndrome. In a Dutch family with the clinically overlapping Jeune syndrome, a homozygous missense mutation in the same gene was found. Both families displayed a nephronophthisis-like nephropathy. Independently, we also identified compound heterozygous WDR19 mutations by exome sequencing in a Moroccan family with isolated nephronophthisis. WDR19 encodes IFT144, a member of the intraflagellar transport (IFT) complex A that drives retrograde ciliary transport. We show that IFT144 is absent from the cilia of fibroblasts from one of the Sensenbrenner patients and that ciliary abundance and morphology is perturbed, demonstrating the ciliary pathogenesis. Our results suggest that isolated nephronophthisis, Jeune, and Sensenbrenner syndromes are clinically overlapping disorders that can result from a similar molecular cause. PMID:22019273

Specialized Cilia in Mammalian Sensory Systems

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Nathalie Falk

2015-09-01

Full Text Available Cilia and flagella are highly conserved and important microtubule-based organelles that project from the surface of eukaryotic cells and act as antennae to sense extracellular signals. Moreover, cilia have emerged as key players in numerous physiological, developmental, and sensory processes such as hearing, olfaction, and photoreception. Genetic defects in ciliary proteins responsible for cilia formation, maintenance, or function underlie a wide array of human diseases like deafness, anosmia, and retinal degeneration in sensory systems. Impairment of more than one sensory organ results in numerous syndromic ciliary disorders like the autosomal recessive genetic diseases Bardet-Biedl and Usher syndrome. Here we describe the structure and distinct functional roles of cilia in sensory organs like the inner ear, the olfactory epithelium, and the retina of the mouse. The spectrum of ciliary function in fundamental cellular processes highlights the importance of elucidating ciliopathy-related proteins in order to find novel potential therapies.

Métodos comparativos para recomposição de áreas de mata ciliar avaliados por análise longitudinal = Comparative methods for ciliary forest rearrangement evaluated by longitudinal analysis

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Alessandra Costa Carrito Bobato

2008-01-01

Full Text Available Foram avaliados três métodos de distribuição de mudas para recomposição artificial de área de mata ciliar estudando-se a variação do crescimento da estatura e diâmetro durante seis períodos em um ano, das espécies Schinus terbinthifolia Raddi–aroeira ePeltophorum dubium Taub–canafístula. Os métodos estudados foram: mudas distribuídas ao acaso em espaçamento de 2 x 2 m; mudas distribuídas individualmente ao acaso; mudas distribuídas ao acaso em agrupamento três a três, com espaçamento de 1 a 1,5 m. O crescimento da estatura e do diâmetro das espécies estudadas está diretamente relacionado com os períodos em estudo, o tratamento de recomposição e as espécies. O tratamento mudas distribuídas ao acaso individualmente foi o que apresentou melhor crescimento médio do diâmetro.This trial aimed at evaluating three methods to distribute seedlingsfrom Schinus terbinthifolia and Peltophorum dubium species for rearrangement of ciliary forests. Their growth changes in height and diameter were used as parameters during six terms in one year. The studied methods were: seedlings randomized distributed in 2 x 2 m; seedlings individually distributed in a randomized design and seedlings randomized distributed in groups by three, using a 1 to 1.5 m row distance. The growth regarding diameter and height of the observed species is straightly related to the studied periods, rearrangement treatment and species. As a conclusion, it was registered that the seedling treatment individually distributed in a randomized design was the best answer regarding the average growth ofdiameter.

A designated centre for people with disabilities operated by Brothers of Charity Services Galway, Galway

LENUS (Irish Health Repository)

Casey, Jillian P

2015-01-01

Primary ciliary dyskinesia (PCD) is a rare autosomal recessive disorder characterised by abnormal ciliary motion and impaired mucociliary clearance, leading to recurrent respiratory infections, sinusitis, otitis media and male infertility. Some patients also have laterality defects. We recently reported the identification of three disease-causing PCD genes in the Irish Traveller population; RSPH4A, DYX1C1 and CCNO. We have since assessed an additional Irish Traveller family with a complex phenotype involving PCD who did not have any of the previously identified PCD mutations.

[Histological structure of the trabecular meshwork in the eyeball: challenging the traditional concept and preliminary findings in rabbits, rats and mice].

Science.gov (United States)

Shi, Yun; Zhou, Fan-Qi; Luo, Zhou-Cai; Chen, Ying-Hua; Chen, Yu; Dong, Wei-Ren

2017-10-20

To verify that the trabecular meshwork (TM) in the wall of the eyeball consists of smooth muscle fibers instead of collagen fibers or endothelial cells. Eighteen fresh eyeballs from 3 rabbits, 3 SD rats and 3 mice were sectioned along the sagittal plane and sliced after paraffin embedding for HE staining, VG staining, Masson staining, α-SMA immunohistochemistry or CD31 immunohistochemistry. These slices were observed under microscope and the structure of the TM was compared with those of scleral collagen fibers, ciliary muscles and endothelial cells. HE staining of the eyeball slices from the 3 animal species resulted in purplish red staining of the TM, which was highly consistent with ciliary muscle fibers. The cell?like structures on the surface of the TM were not clearly outlined, with flat nuclei showing a dark purple staining; these structures did not show obvious boundaries from the TM. Ciliary muscle fibers, which were smooth muscle cells in nature, were aligned in bundles in various directions. The longitudinally sectioned cells were flat and contained purplish cytoplasm and highly flattened nuclei. Scleral collagen fibers were stained dark red with a few fibroblasts sandwiched among them. The long axis of the fibroblasts was in parallel with that of the collagen fibers. The outline of the fibroblast was not clear and the nucleus was flat in dark blue. The vascular endothelial cells presented with different morphologies and contained light purplish cytoplasm and dark nuclei, protruding into the vascular cavity. VG staining of the TM revealed a pale red filamentous structure, and the collagen fibers were stained bright red. Masson staining of the TM showed a reticular structure consisting mainly of dark red fibers intermingled with thin green fibers. Scleral collagen fibers presented with a cord?like green wavy structure. The endothelial cells were green and flat, while the ciliary smooth muscle fibers were purple. In immunohistochemistry for α?SMA, the TM

Effect of cigarette smoking on nasal mucociliary clearance: A comparative analysis using saccharin test

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Manu Kurian Baby

2014-01-01

Full Text Available Background: Nasal mucociliary clearance (NMC system transports the mucus layer covering the nasal epithelium towards nasopharynx by ciliary beating at a frequency of 7-16 Hz. NMC is altered by septal deviations, upper respiratory infections, and drugs. Few studies have revealed significant depression of ciliary activity in smokers. We conducted this study to compare NMC and influence duration of smoking on NMC in adult smokers and nonsmokers using saccharin test. Materials and Methods: Our study included 30 nonsmokers and 30 smokers (21-40 years who were not on any medications and had no history of any systemic illness. Time elapsing until the first experience of sweet taste at posterior nasopharynx, following placement of saccharin particle approximately 1 cm behind the anterior end of inferior turbinate was recorded as NMC time in minutes using standard method described by Anderson. Mean NMC of both groups were compared using Student′s t -test and influence of duration was analyzed by one-way Analysis of variance (ANOVA. Results: NMC was significantly prolonged in smokers (481.2 ± 29.83; P 5 years = 637.5 ± 28.49 s; F statistic = 20.8968, P = 0.0000. Conclusions: NMC measurement is a simple and useful index for the assessment of effect of smoking on the ciliary activity of respiratory mucosa. Prolonged clearance observed in smokers of our study may be due to slowed ciliary beat frequency or reduction in number of cilia and changes in viscoelastic properties of mucus.

Functional Architecture of the Outer Arm Dynein Conformational Switch*

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King, Stephen M.; Patel-King, Ramila S.

2012-01-01

Dynein light chain 1 (LC1/DNAL1) is one of the most highly conserved components of ciliary axonemal outer arm dyneins, and it associates with both a heavy chain motor unit and tubulin located within the A-tubule of the axonemal outer doublet microtubules. In a variety of model systems, lack of LC1 or expression of mutant forms leads to profound defects in ciliary motility, including the failure of the hydrodynamic coupling needed for ciliary metachronal synchrony, random stalling during the power/recovery stroke transition, an aberrant response to imposed viscous load, and in some cases partial failure of motor assembly. These phenotypes have led to the proposal that LC1 acts as part of a mechanical switch to control motor function in response to alterations in axonemal curvature. Here we have used NMR chemical shift mapping to define the regions perturbed by a series of mutations in the C-terminal domain that yield a range of phenotypic effects on motility. In addition, we have identified the subdomain of LC1 involved in binding microtubules and characterized the consequences of an Asn → Ser alteration within the terminal leucine-rich repeat that in humans causes primary ciliary dyskinesia. Together, these data define a series of functional subdomains within LC1 and allow us to propose a structural model for the organization of the dynein heavy chain-LC1-microtubule ternary complex that is required for the coordinated activity of dynein motors in cilia. PMID:22157010

Immotile cilia syndrome in an aged dog

International Nuclear Information System (INIS)

Killingsworth, C.R.; Slocombe, R.F.; Wilsman, N.J.

1987-01-01

An 11-year-old Dalmatian was examined and treated for bilateral nasal discharge and cough of 6 months' duration. Response to medical treatment and surgical intervention was unsatisfactory. Histologic examination of lung tissue revealed chronic severe catarrhal bronchitis and bronchiolitis with bronchiectasis. Histologic findings and barium sulfate bronchography indicated abnormal mucociliary clearance in the respiratory tract. Electron microscopy revealed abnormalities or deletions of outer and/or inner dynein arms in 26% of the ciliary profiles from the affected dog. Similar abnormalities were not found in 500 ciliary profiles from age- and gender-matched control dogs

Pharmacological characterization of NMDA-like receptors in the single-celled organism Paramecium primaurelia.

Science.gov (United States)

Ramoino, Paola; Candiani, Simona; Pittaluga, Anna Maria; Usai, Cesare; Gallus, Lorenzo; Ferrando, Sara; Milanese, Marco; Faimali, Marco; Bonanno, Giambattista

2014-02-01

Paramecium primaurelia is a unicellular eukaryote that moves in freshwater by ciliary beating and responds to environmental stimuli by altering motile behaviour. The movements of the cilia are controlled by the electrical changes of the cell membrane: when the intraciliary Ca(2+) concentration associated with plasma membrane depolarization increases, the ciliary beating reverses its direction, and consequently the swimming direction changes. The ciliary reversal duration is correlated with the amount of Ca(2+) influx. Here, we evaluated the effects due to the activation or blockade of N-methyl-d-aspartic acid (NMDA) receptors on swimming behaviour in Paramecium. Paramecia normally swim forward, drawing almost linear tracks. We observed that the simultaneous administration of NMDA and glycine induced a partial ciliary reversal (PaCR) leading to a continuous spiral-like swim. Furthermore, the duration of continuous ciliary reversal (CCR), triggered by high external KCl concentrations, was longer in NMDA+glycine-treated cells. NMDA action required the presence of Ca(2+), as the normal forward swimming was restored when the ion was omitted from the extracellular milieu. The PaCR and the enhancement of CCR duration significantly decreased when the antagonists of the glutamate site D-AP5 or CGS19755, the NMDA channel blocker MK-801 or the glycine site antagonist DCKA was added. The action of NMDA+glycine was also abolished by Zn(2+) or ifenprodil, the GluN2A and the GluN2B NMDA-containing subunit blockers, respectively. Searches of the Paramecium genome database currently available indicate that the NMDA-like receptor with ligand-binding characteristics of an NMDA receptor-like complex, purified from rat brain synaptic membranes and found in some metazoan genomes, is also present in Paramecium. These results provide evidence that functional NMDA receptors similar to those typical of mammalian neuronal cells are present in the single-celled organism Paramecium and thus

Mucosal pathology of an experimental otitis media with effusion after X-ray irradiation

International Nuclear Information System (INIS)

Ohashi, Y.; Nakai, Y.; Ikeoka, H.; Esaki, Y.; Koshimo, H.; Onoyama, Y.

1987-01-01

Ten guinea pigs were irradiated with 30 Gy of x-radiation. Five were killed on the eighth day after irradiation, and the remainder were killed at the sixteenth day after irradiation. At the time of death, examination was made of the ciliary activity and the fine structure of the middle ear mucosa. Serous effusion was found in each tympanic cavity of all animals. It was shown also that the guinea pig, when irradiated with 30 Gy of x-radiation, exhibits pathologic abnormalities similar to those in humans with otitis media with effusion: degeneration of cilia or ciliated cells and changes in the vascular system (capillary injury and increased capillary permeability). Functional examinations showed that x-ray irradiation has delayed effects on ciliary activity, and the effects are much greater at the sixteenth day than at the eighth day. We speculate that the accumulation of effusion can be, at least partially, a consequence of ciliary dysfunction. The induction of sterile effusion by the use of x-ray irradiation provides a unique animal model for chronic otitis media with effusion of the serous type

Kartagener's Syndrome.

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Dhar, D K; Ganguly, K C; Alam, S; Hossain, A; Sarker, U K; Das, B K; Haque, M J

2009-01-01

Kartagener's Syndrome or Immotile Cilia Syndrome, a variant of Primary Ciliary Dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defect in the tiny hair like structure, the cilia lining the respiratory tract (upper and lower), sinuses, eustachian tubes, middle ear and fallopian tubes. Here electron microscopy shows abnormal arrangement of ciliary tubules and patients with Kartagener's syndrome has an absence of dynein arms at the base of the cilia. The inability of cilia to move results in inadequate clearance of bacteria from the air passages, resulting in an increased risk of infection and causing bronchiectasis. Another result of ciliary immobility is infertility. A 60 years old lady was diagnosed as a case of Kartagener's syndrome. She had history of chronic cough for 20 years, irregular fever for 20 years and occasional shortness of breath for 5 years. Relevant investigations revealed dextrocardia, situs inversus, bilateral maxillary sinusitis with non pneumatised frontal sinus and bronchiectasis. She was treated with low concentration oxygen inhalation, antibiotic, bronchodilator, chest physiotherapy including postural drainage, vitamins and other supportive treatment.

Neuropeptide Y family receptors traffic via the Bardet-Biedl syndrome pathway to signal in neuronal primary cilia.

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Loktev, Alexander V; Jackson, Peter K

2013-12-12

Human monogenic obesity syndromes, including Bardet-Biedl syndrome (BBS), implicate neuronal primary cilia in regulation of energy homeostasis. Cilia in hypothalamic neurons have been hypothesized to sense and regulate systemic energy status, but the molecular mechanism of this signaling remains unknown. Here, we report a comprehensive localization screen of 42 G-protein-coupled receptors (GPCR) revealing seven ciliary GPCRs, including the neuropeptide Y (NPY) receptors NPY2R and NPY5R. We show that mice modeling BBS disease or obese tubby mice fail to localize NPY2R to cilia in the hypothalamus and that BBS mutant mice fail to activate c-fos or decrease food intake in response to the NPY2R ligand PYY3-36. We find that cells with ciliary NPY2R show augmented PYY3-36-dependent cAMP signaling. Our data demonstrate that ciliary targeting of NPY receptors is important for controlling energy balance in mammals, revealing a physiologically defined ligand-receptor pathway signaling within neuronal cilia. Copyright © 2013 The Authors. Published by Elsevier Inc. All rights reserved.

Distribution of CD163-positive cell and MHC class II-positive cell in the normal equine uveal tract.

Science.gov (United States)

Sano, Yuto; Matsuda, Kazuya; Okamoto, Minoru; Takehana, Kazushige; Hirayama, Kazuko; Taniyama, Hiroyuki

2016-02-01

Antigen-presenting cells (APCs) in the uveal tract participate in ocular immunity including immune homeostasis and the pathogenesis of uveitis. In horses, although uveitis is the most common ocular disorder, little is known about ocular immunity, such as the distribution of APCs. In this study, we investigated the distribution of CD163-positive and MHC II-positive cells in the normal equine uveal tract using an immunofluorescence technique. Eleven eyes from 10 Thoroughbred horses aged 1 to 24 years old were used. Indirect immunofluorescence was performed using the primary antibodies CD163, MHC class II (MHC II) and CD20. To demonstrate the site of their greatest distribution, positive cells were manually counted in 3 different parts of the uveal tract (ciliary body, iris and choroid), and their average number was assessed by statistical analysis. The distribution of pleomorphic CD163- and MHC II-expressed cells was detected throughout the equine uveal tract, but no CD20-expressed cells were detected. The statistical analysis demonstrated the distribution of CD163- and MHC II-positive cells focusing on the ciliary body. These results demonstrated that the ciliary body is the largest site of their distribution in the normal equine uveal tract, and the ciliary body is considered to play important roles in uveal and/or ocular immune homeostasis. The data provided in this study will help further understanding of equine ocular immunity in the normal state and might be beneficial for understanding of mechanisms of ocular disorders, such as equine uveitis.

Wavefront Derived Refraction and Full Eye Biometry in Pseudophakic Eyes.

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Xinjie Mao

Full Text Available To assess wavefront derived refraction and full eye biometry including ciliary muscle dimension and full eye axial geometry in pseudophakic eyes using spectral domain OCT equipped with a Shack-Hartmann wavefront sensor.Twenty-eight adult subjects (32 pseudophakic eyes having recently undergone cataract surgery were enrolled in this study. A custom system combining two optical coherence tomography systems with a Shack-Hartmann wavefront sensor was constructed to image and monitor changes in whole eye biometry, the ciliary muscle and ocular aberration in the pseudophakic eye. A Badal optical channel and a visual target aligning with the wavefront sensor were incorporated into the system for measuring the wavefront-derived refraction. The imaging acquisition was performed twice. The coefficients of repeatability (CoR and intraclass correlation coefficient (ICC were calculated.Images were acquired and processed successfully in all patients. No significant difference was detected between repeated measurements of ciliary muscle dimension, full-eye biometry or defocus aberration. The CoR of full-eye biometry ranged from 0.36% to 3.04% and the ICC ranged from 0.981 to 0.999. The CoR for ciliary muscle dimensions ranged from 12.2% to 41.6% and the ICC ranged from 0.767 to 0.919. The defocus aberrations of the two measurements were 0.443 ± 0.534 D and 0.447 ± 0.586 D and the ICC was 0.951.The combined system is capable of measuring full eye biometry and refraction with good repeatability. The system is suitable for future investigation of pseudoaccommodation in the pseudophakic eye.

[Spasm of accommodation].

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Lindberg, Laura

2014-01-01

Spasm of accommodation refers to prolonged contraction of the ciliary muscle, most commonly causing pseudomyopia to varying degrees in both eyes by keeping the lens in a state of short sightedness. It may also be manifested as inability to allow the adaptation spasticity prevailing in the ciliary muscle relax without measurable myopia. As a rule, this is a functional ailment triggered by prolonged near work and stress. The most common symptoms include blurring of distance vision, varying visual acuity as well as pains in the orbital region and the head, progressing into a chronic state. Cycloplegic eye drops are used as the treatment.

A Comparison of Fishes and Invertebrates Living in the Vicinity of Energized and Unenergized Submarine Power Cables and Natural Sea Floor off Southern California, USA

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Milton S. Love

2017-01-01

Full Text Available Increasing reliance on deep-water renewable energy has increased concerns about the effects of the electromagnetic fields (EMFs generated by submarine power cables on aquatic organisms. Off southern California, we conducted surveys of marine organisms living around energized and unenergized submarine power cables and nearby sea floor during 2012–2014 at depths between 76 and 213 m. In general, EMFs declined to background levels about one meter from the cable. We found no statistical difference in species composition between the fish assemblages along the energized and unenergized cables. The natural habitat community statistically differed from both energized and unenergized cable communities. Within species (or species groups, we found no differences in densities between energized and unenergized cables. Total fish densities were significantly higher around the cables than over the natural habitat. We found that invertebrate communities were structured by habitat type and depth and, similar to the fishes, there was no statistical difference between the energized and unenergized cables. Individually, the densities of four invertebrate species or species groups (Metridium farcimen, Luidia spp., unidentified black Crinoidea, and Urticina spp. differed between energized and unenergized cables, but this difference was not significant across all depth strata. The invertebrate community inhabiting the natural habitat strongly differed from the energized and unenergized cable community exhibiting the fewest species and individuals.

Biomicroscopia ultra-sônica (UBM no planejamento cirúrgico e acompanhamento pós-operatório de melanomas malignos de íris e corpo ciliar Ultrasound biomicroscopy (UBM in the surgical planning and postoperative follow-up of iris and ciliary body melanomas

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Zélia Maria da Silva Corrêa

2003-08-01

Full Text Available OBJETIVO: Estudar os achados da biomicroscopia ultra-sônica (UBM em melanomas malignos primários da íris e corpo ciliar antes e depois da resecção cirúrgica e correlacionar a extensão do tumor (revelado pela biomicroscopia ultra-sônica aos achados histopatológicos. MÉTODOS: Estudo prospectivo de 5 pacientes com diagnóstico clínico de tumor de íris e/ou corpo ciliar. Estes pacientes foram avaliados pela biomicroscopia ultra-sônica antes e após iridociclectomia ou iridectomia. Os espécimens foram enviados para estudo histopatológico. Os pacientes foram acompanhados por período mínimo de 6 meses quando foi realizada nova biomicroscopia ultra-sônica. RESULTADOS: Neste grupo de 5 pacientes, 3 eram do sexo feminino e 2, do masculino, variação de idade de 32 e 64 anos. Todos os casos eram unilaterais. Quatro pacientes apresentaram massa iridociliar e um apresentou lesão limitada à íris. Em todos os casos a biomicroscopia ultra-sônica auxiliou o planejamento cirúrgico para a ressecção tumoral com margens livres. A biomicroscopia ultra-sônica no pós-operatório revelou ausência de crescimento tumoral em todos os casos, sendo que 2 pacientes apresentaram vítreo na incisão cirúrgica, 1 deles com tração vítrea leve, mas sem sinais de descolamento de retina. CONCLUSÃO: A biomicroscopia ultra-sônica pareceu ser método auxiliar confiável para diagnosticar tumores de íris e corpo ciliar, planejar o tratamento cirúrgico e o acompanhamento pós-operatório.PURPOSE: To study pre-operatively and post-operatively ultrasound biomicroscopy (UBM findings of iris and ciliary body melanomas and correlate tumor extension (revealed by ultrasound biomicroscopy with histopathology findings. METHODS: Prospective study of 5 patients with a clinical diagnosis of iris and ciliary body tumors. These patients were evaluated by ultrasound biomicroscopy before and after treatment with iridectomy or iridociclectomy. All specimens were sent

Anterior but not posterior choroid changed before and during Valsalva manoeuvre in healthy Chinese: a UBM and SS-OCT study

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Li, Fei; Gao, Kai; Li, Xingyi; Chen, Shida; Huang, Wenbin; Zhang, Xiulan

2017-01-01

Purpose To determine if the anterior choroid is involved in ocular change during the Valsalva manoeuvre (VM). Materials and methods Fifty-three healthy volunteers aged 18–65 years with normal visual field test results and no history of intraocular pressure (IOP) exceeding 21 mm Hg were recruited. Anterior and posterior choroidal changes before and during VM were recorded by ultrasound microscope and swept-source optical coherence tomography, respectively. Parameters of the anterior segment included ciliary body thickness (CBT0), thickness of the choroid at a distance of 4 mm from the root of the iris (CT4), anterior placement of the ciliary body (APCB) and trabecular–ciliary angle (TCA). Thickness of different layers of retina and posterior choroid were also measured and compared before and during VM. IOP, blood pressure (BP), heart rate (HR), axial length, spherical equivalent refractive error and pupil diameter (PD) were also recorded and analysed. Results VM caused elevated IOP, systolic BP, diastolic BP and increased HR. There was a significant increase in anterior parameters including CBT0, CT4 and APCB (p0.05). The mean change of CBT0, CT4 and APCB were: from 1.00±0.09 mm to 1.11±0.10 mm (p<0.001), from 0.29±0.04 mm to 0.36±0.05 mm (p<0.001), from 0.76±0.11 mm to 0.88±0.13 mm (p<0.001), respectively. However, there is no significant change in posterior choroid (from 215.74±60.23 µm to 214.82±61.32 µm, p=0.17). Conclusion We found that VM did not affect the posterior choroid, but it did cause thickening of the anterior choroid and the ciliary body, both of which led to a larger anterior placement of the ciliary body and a narrowed anterior chamber. The anterior (but not the posterior) choroid could be related to IOP elevation and a narrowed anterior chamber in primary angle closure diseases. PMID:28432110

HUBUNAGN KEMAMPUAN PERGANTIAN INANG DENGAN PLASTISITAS GENETIKA PADA CENDAWAN BLAS PADI (PYRICULARIA GRISEA

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Sri Listiyowati

2009-08-01

Full Text Available The Digitaria ciliaris, wild grass grown around rice field, was a host for Pyricularia grisea (Cooke Sacc., the fungi caused blast disease of rice. This fungi have a specific mechanism to regenerate new genetic variation in its life cycle. The aim of this research is to study the relation between the ability of the fungi to infect different species of host with its genetic plasticity. It was used three SCAR molecular markers Cut1, Pwl 1 and Erg2. P. grisea isolates (Dc4J1 originated from D. ciliaris at Jasinga-Bogor were able to infect rice cultivars Kencana Bali and Cisokan. The original Dc4J1, from D. ciliaris, and the Dc4J1 that were reisolated from the infected rice cultivars (reisolates-1 had the same ability to infect Kencana Bali and Cisokan. Molecular technique showed that there was a different molecular marker genotype between the original Dc4J1, from D. ciliaris, and the Dc4J1 reisolated from infected rice cultivars. The original Dc4J1 owned Cut1 but did not Pwl2 in contrary the reisolates Dc4J1 from rice cultivars (reisolates-1 had Pwl2 but did not Cut1. The Erg2 presented in both the original and the reisolated Dc4J1. These results indicated that there were a change of genotype of P. grisea at the same time with the change of host species. The Dc4J1 isolates originated from Kencana Bali and Cisokan (reisolates-2 that were infected by reisolate-1, had the same genotype with the reisolates-1

Cerebral Abscess and Extraaxial Empyema in a Patient with Kartagener Syndrome

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Idil Gunes Tatar

2013-06-01

Full Text Available The triad of situs inversus totalis, bronchiectasis and sinusitis is known as Kartagener syndrome which is among the diseases with ciliopathies. Herein we present a case of cerebral abscess and extraaxial empyema detected in a 21-year-old male patient with Kartagener syndrome and a 10-year history of substance abuse. Preoperative CT, MRI findings and postoperative complications are presented with clinical and radiological review of primary ciliary dyskinesia. The consideration of primary ciliary dyskinesia in the differential diagnosis of frequent occurence of cough, rhinitis and otitis media in children is crucial since early diagnosis is known to affect the short term and long term morbidity.

Lack of Radiation Maculopathy After Palladium-103 Plaque Radiotherapy for Iris Melanoma

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Yousef, Yacoub A. [New York Eye Cancer Center, New York Eye and Ear Infirmary, and New York University School of Medicine, New York, NY (United States); Finger, Paul T., E-mail: pfinger@eyecancer.com [New York Eye Cancer Center, New York Eye and Ear Infirmary, and New York University School of Medicine, New York, NY (United States)

2012-07-15

Purpose: To report on the risk of radiation maculopathy for iris and iridociliary melanomas treated by {sup 103}Pd plaque radiotherapy. Methods and Materials: This is a retrospective clinical case series of 30 eyes in 30 patients with melanomas limited to the iris or invading the ciliary body. The main outcome measures included demographic information, laterality, tumor size, location, visual acuity, radiation dose, local control, retinal evaluation, and duration of follow-up. Results: Thirty patients were followed for a median 36 months (range, 12-90 months). Sixteen of 30 tumors (53%) were pure iris melanomas, and 14 (47%) were primary iris melanomas extending into the ciliary body. Radiation dosimetry showed that the median tumor apex dose was 85 Gy (range, 75-100 Gy), lens dose 43.5 Gy (range, 17.8-60 Gy), fovea dose 1.8 Gy (range, 1.3-5 Gy), and central optic disc dose 1.7 Gy (range, 1.3-4.7 Gy). Cataracts developed in 20 of the 28 phakic eyes (71.4%). No patient in this series developed radiation maculopathy or radiation optic neuropathy. Last best-corrected visual acuity was {>=}20/25 in 28 patients (93%) at a median 36 months' follow-up. Conclusion: Though visual acuities were transiently affected by radiation cataract, no radiation maculopathy or optic neuropathy has been noted after {sup 103}Pd treatment of iris and iridociliary melanomas.

Biomicroscopia ultra-sônica na avaliação da posição das lentes intra-oculares em uma técnica de fixação escleral Ultrasound biomicroscopy in the assessment of the intraocular lenses positioning in a transscleral fixation technique

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Raul de Camargo Vianna Filho

2000-10-01

Full Text Available Objetivo: Avaliar pela biomicrospia ultra-sônica (UBM o posicionamento, em relação ao sulco ciliar, das alças de lentes intra-oculares (LIO, em uma técnica de fixação escleral, avaliando-se também se dois pontos de fixação são suficientes para que não haja inclinação da parte óptica. Métodos: Dezesseis olhos afácicos foram submetidos a implante LIO por uma mesma técnica de fixação escleral, realizados por um mesmo cirurgião. Um mês após a cirurgia, o posicionamento das alças das LIO foram avaliados pelo UBM, assim como distâncias entre as LIO e córnea. Os resultados foram submetidos a testes estatísticos. Resultados: Das 32 alças fixadas à esclera, oito estavam localizadas no sulco ciliar e 24 fora deste. Não houve diferença estatística nas distâncias entre LIO e córnea para alças posicionadas no sulco ciliar quando comparadas àquelas localizadas fora do sulco. Isto sugere que, além da distância ao limbo que se transfixa a esclera, outros fatores devem estar associados ao posicionamento da alça no sulco ciliar. As medidas LIO -- córnea realizadas na periferia das LIO às 3, 6, 9, e 12 horas foram semelhantes, mostrando que dois pontos de fixação são suficientes para que a LIO não fique inclinada. Conclusões: Outros fatores (por exemplo o ângulo de abertura do corpo ciliar, além da distância ao limbo na qual se transfixa a esclera, são importantes para o posicionamento das alças no sulco ciliar. Dois pontos de fixação são suficientes para que a LIO não apresente inclinação dentro do olho.Purpose: To assess, through ultrasound biomicroscopy (UBM, the positioning of the intraocular lenses (IOL haptics as related to the ciliary sulcus by using a scleral fixation technique; and to evaluate, as well, if two fixation points are sufficient to avoid tilting of the lenses. Methods: Sixteen aphakic eyes underwent an IOL implan-tation by means of the same scleral fixation technique, performed by the

Application of ultrasound biomicro-scopy in the planning of cataract surgery in anterior megalophthalmos

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Mohammad Ali Zare

2011-01-01

Full Text Available Anterior megalophthalmos, a rare hereditary disorder, is macrocornea (horizontal corneal diameter more than 13 mm in association with enlarged lens-iris diaphragm and ciliary ring. One of the major challenging issues in the cataract surgery of these patients is preventing intraocular lens (IOL malposition, because of probable large capsular bag. Several approaches have been selected by previous surgeons, such as, custom-made anterior chamber IOLs. In this study, we show a normal capsular bag diameter despite ciliary ring enlargement, with application of ultrasound biomicroscopy (UMB. We suggest that in cases of anterior megalophthalmos without phacodonesis, UBM could measure the actual size of the capsular bag and obviate the need for further procedures.

High frequency ultrasound imaging in pupillary block glaucoma.

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Aslanides, I M; Libre, P E; Silverman, R H; Reinstein, D Z; Lazzaro, D R; Rondeau, M J; Harmon, G K; Coleman, D J

1995-01-01

BACKGROUND--The diagnosis of pupillary block glaucoma requires sufficient clarity of the ocular media. This is particularly important for assessment of both the presence and patency of an iridotomy, and the determination of central anterior chamber depth. METHODS--High frequency ultrasonography was used in three patients with suspected pupillary block to determine iris configuration, posterior chamber volume, and ciliary body conformation. RESULTS--All patients demonstrated high frequency ultrasonographic findings consistent with pupillary block: iris bombé, a formed posterior chamber, and a lack of anterior rotation of the ciliary processes. CONCLUSION--High frequency ultrasound imaging appears to be a valuable adjunct in making or corroborating the diagnosis of pupillary block glaucoma. Images PMID:8534666

Pitchfork and Gprasp2 Target Smoothened to the Primary Cilium for Hedgehog Pathway Activation.

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Bomi Jung

Full Text Available The seven-transmembrane receptor Smoothened (Smo activates all Hedgehog (Hh signaling by translocation into the primary cilia (PC, but how this is regulated is not well understood. Here we show that Pitchfork (Pifo and the G protein-coupled receptor associated sorting protein 2 (Gprasp2 are essential components of an Hh induced ciliary targeting complex able to regulate Smo translocation to the PC. Depletion of Pifo or Gprasp2 leads to failure of Smo translocation to the PC and lack of Hh target gene activation. Together, our results identify a novel protein complex that is regulated by Hh signaling and required for Smo ciliary trafficking and Hh pathway activation.

[Application research of DEI technique based on synchrotron X-ray source in imaging rabbit eyeball in vitro].

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Yin, Hong-xia; Huang, Zhi-feng; Wang, Zhen-chang; Liu, Zhao-hui; Li, Yong; Zhu, Pei-ping

2010-03-23

To study the application of DEI technique in imaging the small structures of rabbit eyeball. DEI technique was used to image the eyeball of New Zealand white rabbit in vitro. The experiments were performed using beamline 4W1A at the topography station of Beijing Synchrotron Radiation Facility (BSRF). DEI image showed clearly the fine structures of the rabbit eyeball, such as the transparent cornea, the sclera, the ciliaris, and the ciliary body. DEI is a new X-ray imaging modality which achieves high contrast and spatial resolution. It also showed obvious effect of edge enhancement. DEI has good potential in observing the micro-structures of eyeballs and other small organs.

Lectin histochemical aspects of mucus function in the oesophagus of the reticulated python (Python reticulatus).

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Meyer, W; Luz, S; Schnapper, A

2009-08-01

Using lectin histochemistry, the study characterizes basic functional aspects of the mucus produced by the oesophageal epithelium of the Reticulated python (Python reticulatus). Reaction staining varied as related to the two epithelium types present, containing goblet cells and ciliary cells. Remarkable intensities were achieved especially in the luminal mucus layer and the fine mucus covering the epithelial ciliary border for Con A (alpha-D-Man; alpha-D-Glc) as part of neutral glycoproteins, Limax flavus agglutinin (NeuNac = NeuNgc), emphasizing that water binding hyaluronan provides a hydrated interface conductive to the passage of material and UEA-I (alpha-L-Fuc), corroborating the view that fucose-rich highly viscous mucus is helpful against mechanical stress during prey transport.

Information Space, Information Field, Information Environment

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Victor Ya. Tsvetkov

2014-08-01

Full Text Available The article analyzes information space, information field and information environment; shows that information space can be natural and artificial; information field is substantive and processual object and articulates the space property; information environment is concerned with some object and acts as the surrounding in relation to it and is considered with regard to it. It enables to define information environment as a subset of information space. It defines its passive description. Information environment can also be defined as a subset of information field. It corresponds to its active description.

Is the use of benzalkonium chloride as a preservative for nasal formulations a safety concern? A cautionary note based on compromised mucociliary transport.

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Bernstein, I L

2000-01-01

Topical nasal solution and suspension delivery systems are available for short- and long-acting vasoconstrictors, ipratropium, cromolyn, azelastine, and glucocorticosteroids. The use of intranasal glucocorticosteroids has increased substantially because the efficacy of these agents has been well established for the treatment of perennial and seasonal allergic rhinitis. Adverse local effects of burning, irritation, and dryness are occasionally associated with glucocorticosteroid nasal preparations. Benzalkonium chloride (BKC) is a quaternary ammonium antimicrobial agent included in some nasal solutions (including glucocorticosteroids) to prevent the growth of bacteria. Some reports suggest that BKC in nasal sprays may cause adverse effects, including reduced mucociliary transport, rhinitis medicamentosa, and neutrophil dysfunction. This article summarizes recent literature about possible adverse biologic effects associated with BKC as a nasal spray preservative by examining its effects on the following properties of mucociliary transport: ciliary motion, ciliary form, ciliary beat frequency, electron microscopy, and particle movement/saccharin clearance tests. Both animal and human in vitro data suggest that BKC promotes ciliostasis and reduction in mucociliary transport that may be partially masked by absorption and dilution effects occurring in respiratory mucus. These possible confounding factors may account for several disparate human in vivo results. The use of BKC-free glucocorticosteroid formulations should be considered, particularly in patients who complain of nasal burning, dryness, or irritation.

Eye lesions in pet birds.

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Tsai, S S; Park, J H; Hirai, K; Itakura, C

1993-03-01

Amongst eye lesions in birds that died in quarantine, cataracts were the most common disorders (37/241, 15.4%), being prevalent in the annular pads of cockatiels (Nymphicus hollandicus), Amazon parrots (Amazona aestiva aestiva) and budgerigars (Melopsittacus undulatus). The incidence in male birds was more than twice that in females. Deposition of crystals, mostly in the cornea, was the second most frequent lesion (21/293, 8.7%), mainly found in cockatiels, parakeets (Psittacula krameri manillensis), Amazon parrots (Amazona aestiva aestiva), budgerigars and finches (Poephila gouldiae gouldiae). These corneal crystals were negative to PAS and Kossa's stains. Six parakeets (Psittacula krameri manillensis) had calcium salts deposited in the inner plexiform layer of the retina and occasionally in the iris and ciliary body. Neither inflammation nor neo-vascularization was observed when cataracts, corneal crystalline deposition, and retinal and ciliary calcification were present. Intranuclear inclusion bodies typical for papovavirus infection were found in the eyelids of six budgerigars (2.5%). Similar inclusions were simultaneously found in the pars ciliaris retinae (4, 1.7%), inner plexiform of retina (1, 0.4%) and anterior epithelium of the cornea (1, 0.4%). Other lesions such as candidial endophthalmitis, conjunctival cryptosporidiosis, corneal dystrophy, keratitis, corneal perforation and iridocyclitis, were occasional findings.

Endoscopic evaluation of therapeutic effects of “Anuloma-Viloma Pranayama” in Pratishyaya w.s.r. to mucociliary clearance mechanism and Bernoulli's principle

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Bhardwaj, Atul; Sharma, Mahendra Kumar; Gupta, Manoj

2013-01-01

The current endeavor intended to evaluate the effectiveness and mode of action of Anuloma-Viloma Pranayama (AVP), i.e., alternate nasal breathing exercise, in resolving clinical features of Pratishyaya, i.e., rhinosinusitis. The present study was directed to validate the use of classical “saccharin test” in measuring the nasal health by measuring mucociliary clearance time. This study also highlights the effects of AVP by application of Bernoulli principle in ventilation of paranasal sinuses and surface oxygenation of nasal and paranasal sinuses ciliary epithelium. Clinically, endoscopically and radiologically diagnosed patients of Pratishyaya, i.e., rhinosinusitis, satisfying the inclusion criteria were selected to perform AVP as a breathing exercise regularly for 30 min every day in order to evaluate the effectiveness of AVP in resolving features of rhinosinusitis. Saccharin test was performed before and after completion of 40 days trial to assess the nasal ciliary activity, which has been proved to be directly related to the health of ciliary epithelium and nasal health overall as well. AVP may be regarded as a catalyst to conspicuously enhance ventilation and oxygenation of the paranasal sinuses and the positively effect the nasal respiratory epithelium by increasing better surface availability of oxygen and negative pressure in the nasal cavity itself. PMID:24696572

RPGR: Its role in photoreceptor physiology, human disease, and future therapies.

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Megaw, Roly D; Soares, Dinesh C; Wright, Alan F

2015-09-01

Mammalian photoreceptors contain specialised connecting cilia that connect the inner (IS) to the outer segments (OS). Dysfunction of the connecting cilia due to mutations in ciliary proteins are a common cause of the inherited retinal dystrophy retinitis pigmentosa (RP). Mutations affecting the Retinitis Pigmentosa GTPase Regulator (RPGR) protein is one such cause, affecting 10-20% of all people with RP and the majority of those with X-linked RP. RPGR is located in photoreceptor connecting cilia. It interacts with a wide variety of ciliary proteins, but its exact function is unknown. Recently, there have been important advances both in our understanding of RPGR function and towards the development of a therapy. This review summarises the existing literature on human RPGR function and dysfunction, and suggests that RPGR plays a role in the function of the ciliary gate, which controls access of both membrane and soluble proteins to the photoreceptor outer segment. We discuss key models used to investigate and treat RPGR disease and suggest that gene augmentation therapy offers a realistic therapeutic approach, although important questions still remain to be answered, while cell replacement therapy based on retinal progenitor cells represents a more distant prospect. Copyright © 2015 The Authors. Published by Elsevier Ltd.. All rights reserved.

Prototapirella ciliates from wild habituated Virunga mountain gorillas (Gorilla beringei beringei) in Rwanda with the descriptions of two new species.

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Ito, Akira; Eckardt, Winnie; Stoinski, Tara S; Gillespie, Thomas R; Tokiwa, Toshihiro

2016-06-01

The morphology of Prototapirella fosseyi n. sp., P. rwanda n. sp. and P. gorillaeImai, Ikeda, Collet, and Bonhomme, 1991 in the Entodiniomorphida were described from the mountain gorillas, Gorilla beringei beringei, in Rwanda. The ciliates have a retractable adoral ciliary zone, four non-retractable ciliary tufts in four caudalia, and one broad skeletal plate beneath the body surface. P. rwanda has a dorsal lobe and ventral lobes in two rows whereas P. fosseyi has no lobes. These two new species have an elongated body, a flat tail flap leaning to the ventral, a macronucleus with a tapering anterior end, a round posterior end and a shallow depression on the dorsal side, a micronucleus lying near the anterior end of macronucleus, a thin left region of the skeletal plate, a distinct skeletal rod plate, and four contractile vacuoles. P. gorillae has some variations in the nuclei and the skeletal plate. The infraciliary bands of three Prototapirella species were the same as some Triplumaria species; a C-shaped adoral polybrachykinety, a slender perivestibular polybrachykinety, and paralabial kineties in their retractable adoral ciliary zone and short lateral polybrachykineties in their four caudalia. The perivestibular polybrachykinety is joined only to the right end of adoral polybrachykinety. Copyright © 2016 Elsevier GmbH. All rights reserved.

Lack of Radiation Maculopathy After Palladium-103 Plaque Radiotherapy for Iris Melanoma

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Yousef, Yacoub A.; Finger, Paul T.

2012-01-01

Purpose: To report on the risk of radiation maculopathy for iris and iridociliary melanomas treated by 103 Pd plaque radiotherapy. Methods and Materials: This is a retrospective clinical case series of 30 eyes in 30 patients with melanomas limited to the iris or invading the ciliary body. The main outcome measures included demographic information, laterality, tumor size, location, visual acuity, radiation dose, local control, retinal evaluation, and duration of follow-up. Results: Thirty patients were followed for a median 36 months (range, 12–90 months). Sixteen of 30 tumors (53%) were pure iris melanomas, and 14 (47%) were primary iris melanomas extending into the ciliary body. Radiation dosimetry showed that the median tumor apex dose was 85 Gy (range, 75–100 Gy), lens dose 43.5 Gy (range, 17.8–60 Gy), fovea dose 1.8 Gy (range, 1.3–5 Gy), and central optic disc dose 1.7 Gy (range, 1.3–4.7 Gy). Cataracts developed in 20 of the 28 phakic eyes (71.4%). No patient in this series developed radiation maculopathy or radiation optic neuropathy. Last best-corrected visual acuity was ≥20/25 in 28 patients (93%) at a median 36 months’ follow-up. Conclusion: Though visual acuities were transiently affected by radiation cataract, no radiation maculopathy or optic neuropathy has been noted after 103 Pd treatment of iris and iridociliary melanomas.

Solving intraocular lens-related pigment dispersion syndrome with repositioning of primary sulcus implanted single-piece IOL in the capsular bag.

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Kohnen, Thomas; Kook, Daniel

2009-08-01

We describe 2 cases of pigment dispersion syndrome (PDS) after uneventful phacoemulsification and implantation of a posterior chamber single-piece intraocular lens (IOL) with a sharp-edge design. In both cases, several days after IOL implantation, marked pigment dispersion was seen on the iris and in the trabecular meshwork, associated with an elevation in intraocular pressure (IOP). Thorough examination showed that the implanted IOL was in the ciliary sulcus. After surgical repositioning of both IOLs in the capsular bag, the pigment dispersion regressed and the IOP returned to normal limits. The 2 cases suggest that particularly in PDS patients, an IOL with an anterior sharp-edge design should be implanted in the capsular bag. Implantation in the ciliary sulcus should be avoided.

Kartagener's syndrome presented with nasal obstruction: A case report

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Suna Asilsoy

2014-08-01

Full Text Available The nasal polyposis is a chronic inflammatory process of the nasal mucosa. Although it is rare in children, there may be also association with cystic fibrosis and primary ciliary dyskinesia. About 50% of primary ciliary dyskinesia patients develop situs inversus and it is known as Kartagener's syndrome. The Kartagener's sydrome is a rare autosomal recessive disorder characterized by sinusitis, bronchiectasis, situs inversus. Clinically, patients present to the otolaryngologist with nasal obstruction. We as pediatricians, should consider nasal polyposis as a rare cause of nasal obstruction in children. In the presence of recurrent upper and lower respiratory tract infections accompanying nasal polyposis, Kartagener's syndrome must be kept in mind as a rare reason. [Cukurova Med J 2014; 39(4.000: 942-945

The CNTF-derived peptide mimetic Cintrofin attenuates spatial-learning deficits in a rat post-status epilepticus model

DEFF Research Database (Denmark)

Russmann, Vera; Seeger, Natalie; Zellinger, Christina

2013-01-01

Ciliary neurotrophic growth factor is considered a potential therapeutic agent for central nervous system diseases. We report first in vivo data of the ciliary neurotrophic growth factor peptide mimetic Cintrofin in a rat post-status epilepticus model. Cintrofin prevented long-term alterations...... in the number of doublecortin-positive neuronal progenitor cells and attenuated the persistence of basal dendrites. In contrast, Cintrofin did neither affect acute status epilepticus-associated alterations in hippocampal cell proliferation and neurogenesis nor reveal any relevant effect on seizure activity....... Whereas status epilepticus caused a significant disturbance in spatial learning in reversed peptide-treated rats, the performance of Cintrofin-treated rats did not differ from controls. The study confirms that Cintrofin comprises an active sequence mimicking effects of its parent molecule. While the data...

Sudden unilateral visual loss after autologous fat injection into the nasolabial fold

Directory of Open Access Journals (Sweden)

Sang Hyouk Park

2008-10-01

Full Text Available Sang Hyouk Park, Hae Jung Sun, Kyung Seek ChoiDepartment of ophthalmology, Soonchunhyang University College of Medicine, Seoul, KoreaAbstract: A 27-year-old female presented with sudden visual loss of her right eye after receiving an autologous fat injection into the right nasolabial fold. Fundus examination of the right eye showed multiple whitish patchy lesions with macular edema. Fluorescein angiogram showed deterioration of choroidal circulation with patchy choroidal filling and arm-to-retina circulation time and retinal arteriovenous passage time were delayed to 30 seconds and 20 seconds, respectively. There was no response in flash visual evoked potential (VEP. High dose steroid therapy (methylprednisolone 1 g/day/i.v. was done and about 2 weeks later, the disc edema subsided and retinal arteriovenous passage time of fluorescein angiogram was normalized but there was no improvement in visual acuity. Absence of a cherry red spot, deterioration of choroidal circulation with patchy choroidal fillings seen in fluorescein angiogram, and no response in flash VEP suggests multiple choroidal infarction due to perfusion defect of the short posterior ciliary artery. The autologous fat injected is thought to have entered the dorsal nasal artery and the retrograde migration of the emboli to the ophthalmic artery might have caused the multiple occlusions of the short posterior ciliary artery.Keywords: autologous fat injection, ciliary artery occlusion, ischemic optic neuropathy

Ofd1 controls dorso-ventral patterning and axoneme elongation during embryonic brain development.

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Anna D'Angelo

Full Text Available Oral-facial-digital type I syndrome (OFDI is a human X-linked dominant-male-lethal developmental disorder caused by mutations in the OFD1 gene. Similar to other inherited disorders associated to ciliary dysfunction OFD type I patients display neurological abnormalities. We characterized the neuronal phenotype that results from Ofd1 inactivation in early phases of mouse embryonic development and at post-natal stages. We determined that Ofd1 plays a crucial role in forebrain development, and in particular, in the control of dorso-ventral patterning and early corticogenesis. We observed abnormal activation of Sonic hedgehog (Shh, a major pathway modulating brain development. Ultrastructural studies demonstrated that early Ofd1 inactivation results in the absence of ciliary axonemes despite the presence of mature basal bodies that are correctly orientated and docked. Ofd1 inducible-mediated inactivation at birth does not affect ciliogenesis in the cortex, suggesting a developmental stage-dependent role for a basal body protein in ciliogenesis. Moreover, we showed defects in cytoskeletal organization and apical-basal polarity in Ofd1 mutant embryos, most likely due to lack of ciliary axonemes. Thus, the present study identifies Ofd1 as a developmental disease gene that is critical for forebrain development and ciliogenesis in embryonic life, and indicates that Ofd1 functions after docking and before elaboration of the axoneme in vivo.

Purification and characterization of factors produced by Aspergillus fumigatus which affect human ciliated respiratory epithelium.

Science.gov (United States)

Amitani, R; Taylor, G; Elezis, E N; Llewellyn-Jones, C; Mitchell, J; Kuze, F; Cole, P J; Wilson, R

1995-09-01

The mechanisms by which Aspergillus fumigatus colonizes the respiratory mucosa are unknown. Culture filtrates of eight of nine clinical isolates of A. fumigatus slowed ciliary beat frequency and damaged human respiratory epithelium in vitro. These changes appeared to occur concurrently. Culture filtrates of two clinical isolates of Candida albicans had no effect on ciliated epithelium. We have purified and characterized cilioinhibitory factors of a clinical isolate of A. fumigatus. The cilioinhibitory activity was heat labile, reduced by dialysis, and partially extractable into chloroform. The activity was associated with both high- and low-molecular-weight factors, as determined by gel filtration on Sephadex G-50. A low-molecular-weight cilioinhibitory factor was further purified by reverse-phase high-performance liquid chromatography and shown by mass spectrometry to be gliotoxin, a known metabolite of A. fumigatus. Gliotoxin significantly slowed ciliary beat frequency in association with epithelial damage at concentrations above 0.2 microgram/ml; other Aspergillus toxins, i.e., fumagillin and helvolic acid, were also cilioinhibitory but at much higher concentrations. High-molecular-weight (> or = 35,000 and 25,000) cilioinhibitory materials had neither elastolytic nor proteolytic activity and remain to be identified. Thus, A. fumigatus produces a number of biologically active substances which slow ciliary beating and damage epithelium and which may influence colonization of the airways.

Asteroidea de la plataforma continental de Rio Grande do Sul (Brasil, coleccionados durante los viajes del N/Oc. "prof. W. Besnard" para el proyecto Rio Grande do Sul Asteroidea of the continental shelf of Rio Grande do Sul (Brazil, collected during the cruises of the R/V "prof. W. Besnard" for the project Rio Grande do Sul

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Carlos J Carrera-Rodríguez

1977-01-01

, Mediaster trindadensis Bernasconi, Sclerasterias subangülosa (Verrill y Tosia parva (Perrier siendo nuevas ocurrencias para el área de estudio y Luidla barbadensis Perrier y L. elegans Perrier siendo nuevas ocurrencias para Brasil. 9 - El género Pectinidiscus Ludwig se reporta por la primera vez para las Américas y se describe una nueva especie: Pectinidiscus aliciae. Se compara con las otras dos especies conocidas del género, P. annae y P. sibogae.The Asteroidea collected during the cruises of the R/V "Prof. W. Besnard" along the coast of Rio Grande do Sul (Brazil from April 1968 to January 1972 were studied. Twenty-four species are reported from the continental shelf for the area between Torres (29ºS and Chui (34ºS. Labidiaster radiosus Lutken, Luidia clathrata (Say, Marginaster pectinatus Perrier, Mediaster trindadensis Bernasconi, Sclerasterias subangulosa (Verrill and Tosia parva (Perrier are new records for the area; Luidia barbadensis Perrier and L. elegans Perrier are new records for Brazil and Pectinidiscus aliciae sp. n. constitutes the first record of the genus from the Americas and is compared with the other two known species of the genus: P. annae and P. sibogae. Ecological data and the geographic distribution for each species are given. Keys for identification are provided. The general hydrology of the area and the biogeography are discussed and correlations of asteroid abundance with two commercially important fish species are made.

The Ciliopathy Protein CC2D2A Associates with NINL and Functions in RAB8-MICAL3-Regulated Vesicle Trafficking.

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Ruxandra Bachmann-Gagescu

2015-10-01

Full Text Available Ciliopathies are a group of human disorders caused by dysfunction of primary cilia, ubiquitous microtubule-based organelles involved in transduction of extra-cellular signals to the cell. This function requires the concentration of receptors and channels in the ciliary membrane, which is achieved by complex trafficking mechanisms, in part controlled by the small GTPase RAB8, and by sorting at the transition zone located at the entrance of the ciliary compartment. Mutations in the transition zone gene CC2D2A cause the related Joubert and Meckel syndromes, two typical ciliopathies characterized by central nervous system malformations, and result in loss of ciliary localization of multiple proteins in various models. The precise mechanisms by which CC2D2A and other transition zone proteins control protein entrance into the cilium and how they are linked to vesicular trafficking of incoming cargo remain largely unknown. In this work, we identify the centrosomal protein NINL as a physical interaction partner of CC2D2A. NINL partially co-localizes with CC2D2A at the base of cilia and ninl knockdown in zebrafish leads to photoreceptor outer segment loss, mislocalization of opsins and vesicle accumulation, similar to cc2d2a-/- phenotypes. Moreover, partial ninl knockdown in cc2d2a-/- embryos enhances the retinal phenotype of the mutants, indicating a genetic interaction in vivo, for which an illustration is found in patients from a Joubert Syndrome cohort. Similar to zebrafish cc2d2a mutants, ninl morphants display altered Rab8a localization. Further exploration of the NINL-associated interactome identifies MICAL3, a protein known to interact with Rab8 and to play an important role in vesicle docking and fusion. Together, these data support a model where CC2D2A associates with NINL to provide a docking point for cilia-directed cargo vesicles, suggesting a mechanism by which transition zone proteins can control the protein content of the ciliary

Inform@ed space

DEFF Research Database (Denmark)

Bjerrum, Peter; Olsen, Kasper Nefer

2001-01-01

Inform@ed space Sensorial Perception And Computer Enchancement - bidrag til Nordisk Arkitekturforskningsforenings IT-konference, AAA april 2001.......Inform@ed space Sensorial Perception And Computer Enchancement - bidrag til Nordisk Arkitekturforskningsforenings IT-konference, AAA april 2001....

Functions of vitamin D / Calcium

Indian Academy of Sciences (India)

First page Back Continue Last page Overview Graphics. Excitation-contraction coupling,. Cardiac functions. Hormonal secretion. Control of enzymatic reactions. Mitotic division. Maintenance of cell integrity. Ciliary motility. Notes: Calcium is a vital second messenger.

Flow of Aqueous Humor

Science.gov (United States)

... Home Flow of Aqueous Humor Flow of Aqueous Humor Most, but not all, forms of glaucoma are ... remains normal when some of the fluid (aqueous humor) produced by the eye's ciliary body flows out ...

Nuevas citas de monocotiledóneas adventicias para la Argentina New records of adventitious monocots for Argentina

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Julio A. Hurrell

2009-12-01

Full Text Available Este trabajo incluye cinco nuevos registros de monocotiledóneas adventicias para la Argentina: Aloe ciliaris Haw. (Asphodelaceae, Aspidistra elatior Blume (Convallariaceae, Sansevieria trifasciata Prain (Dracaenaceae, Phormium tenax J. R. Forst. & G. Forst. (Hemerocallidaceae y Ornithogalum arabicum L. (Hyacinthaceae, pertenecientes al orden Asparagales. También incluye una evaluación del estado actual de estas especies, en relación al proceso de naturalización: escapadas de cultivo ocasionales, naturalizadas.This paper includes five new records of adventitious monocots for Argentina: Aloe ciliaris Haw. (Asphodelaceae, Aspidistra elatior Blume (Convallariaceae, Sansevieria trifasciata Prain (Dracaenaceae, Phormium tenax J. R. Forst. & G. Forst. (Hemerocallidaceae and Ornithogalum arabicum L. (Hyacinthaceae, belonging to order Asparagales. Also includes an evaluation of its status in the naturalization process: casual alien, naturalized.

Molecular mechanisms of water transport in the eye

DEFF Research Database (Denmark)

Hamann, Steffen

2002-01-01

The four major sites for ocular water transport, the corneal epithelium and endothelium, the ciliary epithelium, and the retinal pigment epithelium, are reviewed. The cornea has an inherent tendency to swell, which is counteracted by its two surface cell layers, the corneal epithelium...... and endothelium. The bilayered ciliary epithelium secretes the aqueous humor into the posterior chamber, and the retinal pigment epithelium transports water from the retinal to the choroidal site. For each epithelium, ion transport mechanisms are associated with fluid transport, but the exact molecular coupling...... sites between ion and water transport remain undefined. In the retinal pigment epithelium, a H+-lactate cotransporter transports water. This protein could be the site of coupling between salt and water in this epithelium. The distribution of aquaporins does not suggest a role for these proteins...

On ciliary pumping and sieving in bryozoans

DEFF Research Database (Denmark)

Larsen, Poul Scheel; Riisgård, H. U.

2002-01-01

to be transferred to and conveyed by the frontal cilia bands on tentacles. The added load on laterofrontal cilia from viscous drag on a food particle retained by the cilia is found to be of the same order of magnitude or greater than the "background load" of viscous drag from fluid passing the laterofrontal cilia...... flicks, a description is given of the feeding mechanisms. Particles entering the central region are brought to the mouth by the high velocity feeding current in the central part of the lophophore. Particles entering further out either escape between tentacles or are stopped by the laterofrontal cilia...... sieve and, in the distal region of tentacles, are brought back into the central feeding current by flicks of tentacles. The relative velocity between fluid and particle during a flick recovery phase ensures particle release. Particles stopped in the proximal region of the lophophore appear...

The intraflagellar transport machinery in ciliary signaling

DEFF Research Database (Denmark)

Mourão, André; Christensen, Søren Tvorup; Lorentzen, Esben

2016-01-01

Cilia and flagella on eukaryotic cells are slender microtubule-based projections surrounded by a membrane with a unique lipid and protein composition. It is now appreciated that cilia in addition to their established roles in motility also constitute hubs for cellular signaling by sensing external...

Effect of Cilia Beat Frequency on Mucociliary Clearance

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Sedaghat M. H.

2016-12-01

Full Text Available Background: The airway surface liquid (ASL, which is a fluid layer coating the interior epithelial surface of the bronchi and bronchiolesis, plays an important defensive role against foreign particles and chemicals entering lungs. Objective: Numerical investigation has been employed to solve two-layer model consisting of mucus layer as a viscoelastic fluid and periciliary liquid layer as a Newtonian fluid to study the effects of cilia beat frequency (CBF at various amounts of mucus properties on muco-ciliary transport problem. Methods: Hybrid finite difference-lattice Boltzmann-method (FB-LBM has been used to solve the momentum equations and to simulate cilia forces, and also the PCLmucus interface more accurately, immersed boundary method (IBM has been employed. The main contribution of the current study is to use an Oldroyd-B model as the constitutive equation of mucus. Results: Our results show that increasing CBF and decreasing mucus viscosity ratio have great effects on mucus flow, but the effect of viscosity ratio is more significant. The results also illustrate that the relation between cilia beat frequency and mean mucus velocity is almost linear and it has similar behavior at different values of viscosity ratio. Conclusion: Numerical investigation based on hybrid IB-FD-LBM has been used to study the effect of CBF at various mounts of mucus viscosity ratio on the muco-ciliary clearance. The results showed that the effect of viscosity ratio on the muco-ciliary transport process is more significant compared with CBF.

The primary cilium as a multiple cellular signaling scaffold in development and disease

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Hyuk Wan Ko*

2012-08-01

Full Text Available Primary cilia, single hair-like appendage on the surface of themost mammalian cells, were once considered to be vestigialcellular organelles for a past century because of their tinystructure and unknown function. Although they lack ancestralmotility function of cilia or flagella, they share common groundwith multiciliated motile cilia and flagella on internal structuresuch as microtubule based nine outer doublets nucleated from thebase of mother centrioles called basal body. Making cilia,ciliogenesis, in cells depends on the cell cycle stage due to reuseof centrioles for cell division forming mitotic spindle pole (Mphase and assembling cilia from basal body (starting G1 phaseand maintaining most of interphase. Ciliary assembly requiredtwo conflicting processes such as assembly and disassembly andbalance between these two processes determines the length ofcilia. Both process required highly conserved transport system tosupply needed substance to grow tip of cilia and bring ciliaryturnover product back to the base of cilia using motor protein,kinesin and dynein, and transport protein complex, IFT particles.Disruption of ciliary structure or function causes multiple humandisorder called ciliopathies affecting disease of diverse ciliatedtissues ranging from eye, kidney, respiratory tract and brain.Recent explosion of research on the primary cilia and theirinvolvement on animal development and disease attracts scientificinterest on how extensively the function of cilia related to specificcell physiology and signaling pathway. In this review, I introducegeneral features of primary cilia and recent progress inunderstanding of the ciliary length control and signaling pathwaystransduced through primary cilia in vertebrates.

Morphology and accommodative function of the vitreous zonule in human and monkey eyes.

Science.gov (United States)

Lütjen-Drecoll, Elke; Kaufman, Paul L; Wasielewski, Rainer; Ting-Li, Lin; Croft, Mary Ann

2010-03-01

To explore the attachments of the posterior zonule and vitreous in relation to accommodation and presbyopia in monkeys and humans. Novel scanning electron microscopy (SEM) and ultrasound biomicroscopy (UBM) techniques were used to visualize the anterior, intermediate, and posterior vitreous zonule and their connections to the ciliary body, vitreous membrane, lens capsule, and ora serrata, and to characterize their age-related changes and correlate them with loss of accommodative forward movement of the ciliary body. alpha-Chymotrypsin was used focally to lyse the vitreous zonule and determine the effect on movement of the accommodative apparatus in monkeys. The vitreous attached to the peripheral lens capsule and the ora serrata directly. The pars plana zonule and the posterior tines of the anterior zonule were separated from the vitreous membrane except for strategically placed attachments, collectively termed the vitreous zonule, that may modulate and smooth the forward and backward movements of the entire system. Age-dependent changes in these relationships correlated significantly with loss of accommodative amplitude. Lysis of the intermediate vitreous zonule partially restored accommodative movement. The vitreous zonule system may help to smoothly translate to the lens the driving forces of accommodation and disaccommodation generated by the ciliary muscle, while maintaining visual focus and protecting the lens capsule and ora serrata from acute tractional forces. Stiffening of the vitreous zonular system may contribute to age-related loss of accommodation and offer a therapeutic target for presbyopia.

Resource partitioning between sympatric starfish from tropical unconsolidated substrate: Implications for coexistence and top-down control on benthic prey

Science.gov (United States)

Fernandez, Wellington S.; Dias, Gustavo M.; Majer, Alessandra P.; Delboni, Cynthia G.; Denadai, Marcia R.; Turra, Alexander

2017-09-01

Starfish are important predators that may shape rocky shore communities, but their ecological role in unconsolidated substrate communities is still poorly known. We assessed the feeding niche overlap of two sympatric starfish, Astropecten marginatus and Luidia senegalensis, from the shallow subtidal zone in southeastern Brazil. During one year, we conducted monthly samples to compare diet composition, abundance and frequency of occurrence of each food item between species. With 24 of the 34 food items identified in this study consumed by both species, they exhibited generalist behaviors, with a more diverse diet during the warm periods, when the main prey items were abundant. However, A. marginatus showed more variation in abundance of prey consumed over time than L. senegalensis. The diet of A. marginatus consisted primarily of the bivalve Tivela mactroides and L. senegalensis of the bivalve Mulinia cleryana. The size of T. mactroides was positively correlated to the size of A. marginatus, while only small-sized individuals of L. senegalensis consumed this item, the most abundant prey in the area and an important food resource for local the community. The large quantity and variety of items consumed by both species support the structuring role of starfish in subtidal unconsolidated substrate communities, exerting a generalist top-down control, primarily on dominant bivalve populations. Temporal variation in the availability of the main prey may change how selective are both species. The differences in prey composition between species and the ontogenetic differences in prey selectivity by L. senegalensis may attenuate interspecific competition, facilitating their coexistence.

Paramecium swimming in capillary tube

Science.gov (United States)

Jana, Saikat; Um, Soong Ho; Jung, Sunghwan

2012-04-01

Swimming organisms in their natural habitat need to navigate through a wide range of geometries and chemical environments. Interaction with boundaries in such situations is ubiquitous and can significantly modify the swimming characteristics of the organism when compared to ideal laboratory conditions. We study the different patterns of ciliary locomotion in glass capillaries of varying diameter and characterize the effect of the solid boundaries on the velocities of the organism. Experimental observations show that Paramecium executes helical trajectories that slowly transition to straight lines as the diameter of the capillary tubes decreases. We predict the swimming velocity in capillaries by modeling the system as a confined cylinder propagating longitudinal metachronal waves that create a finite pressure gradient. Comparing with experiments, we find that such pressure gradient considerations are necessary for modeling finite sized ciliary organisms in restrictive geometries.

Accommodation training in foreign workers.

Science.gov (United States)

Takada, Masumi; Miyao, Masaru; Matsuura, Yasuyuki; Takada, Hiroki

2013-01-01

By relaxing the contracted focus-adjustment muscles around the eyeball, known as the ciliary and extraocular muscles, the degree of pseudomyopia can be reduced. This understanding has led to accommodation training in which a visual target is presented in stereoscopic video clips. However, it has been pointed out that motion sickness can be induced by viewing stereoscopic video clips. In Measurement 1 of the present study, we verified whether the new 3D technology reduced the severity of motion sickness in accordance with stabilometry. We then evaluated the short-term effects of accommodation training using new stereoscopic video clips on foreign workers (11 females) suffering from eye fatigue in Measurement 2. The foreign workers were trained for three days. As a result, visual acuity was statistically improved by continuous accommodation training, which will help promote ciliary muscle stretching.

Aquaporins 6-12 in the human eye

DEFF Research Database (Denmark)

Tran, Thuy Linh; Bek, Toke; Holm, Lars

2012-01-01

Purpose: Aquaporins (AQPs) are widely expressed and have diverse distribution patterns in the eye. AQPs 0-5 have been localized at the cellular level in human eyes. We investigated the presence of the more recently discovered AQPs 6-12 in the human eye. Methods: RT-PCR was performed on fresh tissue...... from two human eyes divided into the cornea, corneal limbus, ciliary body and iris, lens, choroid, optic nerve, retina and sclera. Each structure was examined to detect the mRNA of AQPs 6-12. Twenty-one human eyes were examined using immunohistochemical and immunofluorescence techniques to determine...... was detected in the corneal epithelium, corneal endothelium, trabecular meshwork endothelium, ciliary epithelia, lens epithelium, the inner and outer limiting membrane of the retina, the retinal pigment epithelium and the capillary endothelium of all parts of the eye. AQP9 immunolabelling was detected...

Cyclooxygenase-2 expression in the normal human eye and its expression pattern in selected eye tumours

DEFF Research Database (Denmark)

Wang, Jinmei; Wu, Yazhen; Heegaard, Steffen

2011-01-01

Purpose: Cyclooxygenase-2 (COX-2) is an enzyme involved in neoplastic processes. The purpose of the present study is to investigate COX-2 expression in the normal human eye and the expression pattern in selected eye tumours involving COX-2 expressing cells. Methods: Immunohistochemical staining...... using antibodies against COX-2 was performed on paraffin sections of normal human eyes and selected eye tumours arising from cells expressing COX-2. Results: Cyclooxygenase-2 expression was found in various structures of the normal eye. Abundant expression was seen in the cornea, iris, ciliary body...... and retina. The COX-2 expression was less in tumours deriving from the ciliary epithelium and also in retinoblastoma. Conclusion: Cyclooxygenase-2 is constitutively expressed in normal human eyes. The expression of COX-2 is much lower in selected eye tumours involving COX-2 expressing cells....

New Roles of the Primary Cilium in Autophagy.

Science.gov (United States)

Ávalos, Yenniffer; Peña-Oyarzun, Daniel; Budini, Mauricio; Morselli, Eugenia; Criollo, Alfredo

2017-01-01

The primary cilium is a nonmotile organelle that emanates from the surface of multiple cell types and receives signals from the environment to regulate intracellular signaling pathways. The presence of cilia, as well as their length, is important for proper cell function; shortened, elongated, or absent cilia are associated with pathological conditions. Interestingly, it has recently been shown that the molecular machinery involved in autophagy, the process of recycling of intracellular material to maintain cellular and tissue homeostasis, participates in ciliogenesis. Cilium-dependent signaling is necessary for autophagosome formation and, conversely, autophagy regulates both ciliogenesis and cilium length by degrading specific ciliary proteins. Here, we will discuss the relationship that exists between the two processes at the cellular and molecular level, highlighting what is known about the effects of ciliary dysfunction in the control of energy homeostasis in some ciliopathies.

Swimming behavior and prey retention of the polychaete larvae Polydora ciliata (Johnston)

DEFF Research Database (Denmark)

Hansen, B.W.; Jakobsen, Hans Henrik; Andersen, Anders Peter

2010-01-01

in specific feeding rates and the observed increase in the difference between upward and downward swimming speeds with larval size. We estimated a critical larval length above which the buoyancy-corrected weight of the larva exceeds the propulsion force generated by the ciliary swimming apparatus and thus......The behavior of the ubiquitous estuarine planktotrophic spionid polychaete larvae Polydora ciliata was studied. We describe ontogenetic changes in morphology, swimming speed and feeding rates and have developed a simple swimming model using low Reynolds number hydrodynamics. In the model we assumed...... that the ciliary swimming apparatus is primarily composed of the prototroch and secondarily by the telotroch. The model predicted swimming speeds and feeding rates that corresponded well with the measured speeds and rates. Applying empirical data to the model, we were able to explain the profound decrease...

Opacification of the C-flex 570C intraocular lens after sulcus fixation.

Science.gov (United States)

Daigle, Patrick; Carbonneau, Marjorie

2017-09-08

To report a case of diffuse intraocular lens (IOL) opacification in a patient who started complaining of blurred vision in his left eye over the course of three years after having phacoemulsification surgery combined with capsular bag fixation of a C-flex 570C IOL. The IOL had been repositioned in the ciliary sulcus following its subluxation. An IOL exchange was performed, and the explanted IOL was sent for histopathological analysis. Scanning electron microscopy identified multiple crystalline-like deposits on both sides of the IOL. The optic was more extensively involved than the haptics. Energy-dispersive X-ray spectroscopy confirmed the predominance of calcium within the deposits. The breakdown of the blood-aqueous barrier seems to be a key component of this calcification process. Ciliary sulcus fixation is not a suitable option for C-flex 570C IOLs.

Information and Informality

DEFF Research Database (Denmark)

Larsson, Magnus; Segerstéen, Solveig; Svensson, Cathrin

2011-01-01

leaders on the basis of their possession of reliable knowledge in technical as well as organizational domains. The informal leaders engaged in interpretation and brokering of information and knowledge, as well as in mediating strategic values and priorities on both formal and informal arenas. Informal...... leaders were thus seen to function on the level of the organization as a whole, and in cooperation with formal leaders. Drawing on existing theory of leadership in creative and professional contexts, this cooperation can be specified to concern task structuring. The informal leaders in our study...... contributed to task structuring through sensemaking activities, while formal leaders focused on aspects such as clarifying output expectations, providing feedback, project structure, and diversity....

Dexamethasone selectively suppresses microglial trophic responses to hippocampal deafferentation

DEFF Research Database (Denmark)

Woods, A G; Poulsen, F R; Gall, C M

1999-01-01

hippocampus. Daily dexamethasone injections almost completely blocked increases in insulin-like growth factor-1 messenger RNA content, but did not perturb increases in ciliary neurotrophic factor or basic fibroblast growth factor messenger RNA content, in the deafferented dentate gyrus molecular layer...

Beyond information retrieval: information discovery and multimedia information retrieval

OpenAIRE

Roberto Raieli

2017-01-01

The paper compares the current methodologies for search and discovery of information and information resources: terminological search and term-based language, own of information retrieval (IR); semantic search and information discovery, being developed mainly through the language of linked data; semiotic search and content-based language, experienced by multimedia information retrieval (MIR).MIR semiotic methodology is, then, detailed.

Loss-of-function of the ciliopathy protein Cc2d2a disorganizes the vesicle fusion machinery at the periciliary membrane and indirectly affects Rab8-trafficking in zebrafish photoreceptors.

Science.gov (United States)

Ojeda Naharros, Irene; Gesemann, Matthias; Mateos, José M; Barmettler, Gery; Forbes, Austin; Ziegler, Urs; Neuhauss, Stephan C F; Bachmann-Gagescu, Ruxandra

2017-12-01

Ciliopathies are human disorders caused by dysfunction of primary cilia, ubiquitous organelles involved in transduction of environmental signals such as light sensation in photoreceptors. Concentration of signal detection proteins such as opsins in the ciliary membrane is achieved by RabGTPase-regulated polarized vesicle trafficking and by a selective barrier at the ciliary base, the transition zone (TZ). Dysfunction of the TZ protein CC2D2A causes Joubert/Meckel syndromes in humans and loss of ciliary protein localization in animal models, including opsins in retinal photoreceptors. The link between the TZ and upstream vesicle trafficking has been little explored to date. Moreover, the role of the small GTPase Rab8 in opsin-carrier vesicle (OCV) trafficking has been recently questioned in a mouse model. Using correlative light and electron microscopy and live imaging in zebrafish photoreceptors, we provide the first live characterization of Rab8-mediated trafficking in photoreceptors in vivo. Our results support a possibly redundant role for both Rab8a/b paralogs in OCV trafficking, based on co-localization of Rab8 and opsins in vesicular structures, and joint movement of Rab8-tagged particles with opsin. We further investigate the role of the TZ protein Cc2d2a in Rab8-mediated trafficking using cc2d2a zebrafish mutants and identify a requirement for Cc2d2a in the latest step of OCV trafficking, namely vesicle fusion. Progressive accumulation of opsin-containing vesicles in the apical portion of photoreceptors lacking Cc2d2a is caused by disorganization of the vesicle fusion machinery at the periciliary membrane with mislocalization and loss of the t-SNAREs SNAP25 and Syntaxin3 and of the exocyst component Exoc4. We further observe secondary defects on upstream Rab8-trafficking with cytoplasmic accumulation of Rab8. Taken together, our results support participation of Rab8 in OCV trafficking and identify a novel role for the TZ protein Cc2d2a in fusion of incoming

Physiotherapy and bronchial mucus transport

NARCIS (Netherlands)

Schans, Cornelis Peter van der

1991-01-01

The use of physiotherapeutic techniques may increase mucus transport in patients with airways disease including COPD, asthma, cystic fibrosis and primary ciliary dyskinesia. The most effective parts of the treatment are probably forced expirations with open glottis and coughing. However, in patients

Structural and Functional Recovery of Sensory Cilia in C. elegans IFT Mutants upon Aging.

Directory of Open Access Journals (Sweden)

Astrid Cornils

2016-12-01

Full Text Available The majority of cilia are formed and maintained by the highly conserved process of intraflagellar transport (IFT. Mutations in IFT genes lead to ciliary structural defects and systemic disorders termed ciliopathies. Here we show that the severely truncated sensory cilia of hypomorphic IFT mutants in C. elegans transiently elongate during a discrete period of adult aging leading to markedly improved sensory behaviors. Age-dependent restoration of cilia morphology occurs in structurally diverse cilia types and requires IFT. We demonstrate that while DAF-16/FOXO is dispensable, the age-dependent suppression of cilia phenotypes in IFT mutants requires cell-autonomous functions of the HSF1 heat shock factor and the Hsp90 chaperone. Our results describe an unexpected role of early aging and protein quality control mechanisms in suppressing ciliary phenotypes of IFT mutants, and suggest possible strategies for targeting subsets of ciliopathies.

Intestinal cell kinase, a protein associated with endocrine-cerebro-osteodysplasia syndrome, is a key regulator of cilia length and Hedgehog signaling.

Science.gov (United States)

Moon, Heejung; Song, Jieun; Shin, Jeong-Oh; Lee, Hankyu; Kim, Hong-Kyung; Eggenschwiller, Jonathan T; Bok, Jinwoong; Ko, Hyuk Wan

2014-06-10

Endocrine-cerebro-osteodysplasia (ECO) syndrome is a recessive genetic disorder associated with multiple congenital defects in endocrine, cerebral, and skeletal systems that is caused by a missense mutation in the mitogen-activated protein kinase-like intestinal cell kinase (ICK) gene. In algae and invertebrates, ICK homologs are involved in flagellar formation and ciliogenesis, respectively. However, it is not clear whether this role of ICK is conserved in mammals and how a lack of functional ICK results in the characteristic phenotypes of human ECO syndrome. Here, we generated Ick knockout mice to elucidate the precise role of ICK in mammalian development and to examine the pathological mechanisms of ECO syndrome. Ick null mouse embryos displayed cleft palate, hydrocephalus, polydactyly, and delayed skeletal development, closely resembling ECO syndrome phenotypes. In cultured cells, down-regulation of Ick or overexpression of kinase-dead or ECO syndrome mutant ICK resulted in an elongation of primary cilia and abnormal Sonic hedgehog (Shh) signaling. Wild-type ICK proteins were generally localized in the proximal region of cilia near the basal bodies, whereas kinase-dead ICK mutant proteins accumulated in the distal part of bulged ciliary tips. Consistent with these observations in cultured cells, Ick knockout mouse embryos displayed elongated cilia and reduced Shh signaling during limb digit patterning. Taken together, these results indicate that ICK plays a crucial role in controlling ciliary length and that ciliary defects caused by a lack of functional ICK leads to abnormal Shh signaling, resulting in congenital disorders such as ECO syndrome.

MUCOCILIARY DYSFUNCTION IN HIV AND SMOKED SUBSTANCE ABUSE

Directory of Open Access Journals (Sweden)

Srinivasan eChinnapaiyan

2015-10-01

Full Text Available Impaired mucociliary clearance (MCC is a hallmark of acquired chronic airway diseases like chronic bronchitis associated with chronic obstructive pulmonary disease (COPD and asthma. This manifests as microbial colonization of the lung consequently leading to recurrent respiratory infections. People living with HIV demonstrate increased incidence of these chronic airway diseases. Bacterial pneumonia continues to be an important comorbidity in people living with HIV even though anti-retroviral therapy has succeeded in restoring CD4+ cell counts. People living with HIV demonstrate increased microbial colonization of the lower airways. The microbial flora is similar to that observed in diseases like cystic fibrosis and COPD suggesting that mucociliary dysfunction could be a contributing factor to the increased incidence of chronic airway diseases in people living with HIV. The three principal components of the MCC apparatus are, a mucus layer, ciliary beating and a periciliary airway surface liquid (ASL layer that facilitates ciliary beating. Cystic fibrosis transmembrane conductance regulator (CFTR plays a pivotal role in regulating the periciliary airway surface liquid. HIV proteins can suppress all the components of the MCC apparatus by increasing mucus secretion and suppressing CFTR function. This can decrease ASL height leading to suppressed ciliary beating. The effects of HIV on MCC are exacerbated when combined with other aggravating factors like smoking or inhaled substance abuse, which by themselves can suppress one or more components of the MCC system. This review discusses the pathophysiological mechanisms that lead to MCC suppression in people living with HIV who also smoke tobacco or abuse illicit drugs.

The discovery of bioisoster compound for plumbagin using the knowledge-based rational method

Science.gov (United States)

Jeong, Seo Hee; Choi, Jung Sup; Ko, Young Kwan; Kang, Nam Sook

2015-04-01

Arabidopsis thaliana 7-Keto-8-AminoPelargonic Acid Synthase (AtKAPAS) is a crucial herbicide target, and AtKAPAS inhibitors are widely available in the agrochemical market. The herbicide plumbagin is known as a potent inhibitor for AtKAPAS but it is extremely toxic. In this study, we identified the metabolic site of plumbagin and also performed a similarity-based library analysis using 2D fingerprints and a docking study. Four compounds as virtual hits were derived from plumbagin. Treatment of Digitaria ciliaris with compound 2, one of four hit compounds, stunted the growth of leaves and the leaf tissue was desiccated or burned within three days. Thus, we expect that compound 2 will be developed as a new herbicide and additionally our strategy will provide helpful information for optimizing lead compounds.

Information and information flow an introduction

CERN Document Server

Bremer, Manuel

2004-01-01

This book is conceived as an introductory text into the theory of syntactic and semantic information, and information flow. Syntactic information theory is concerned with the information contained in the very fact that some signal has a non-random structure. Semantic information theory is concerned with the meaning or information content of messages and the like. The theory of information flow is concerned with deriving some piece of information from another. The main part will take us to situation semantics as a foundation of modern approaches in information theory. We give a brief overview o

Leukemia inhibitory factor increases glucose uptake in mouse skeletal muscle

DEFF Research Database (Denmark)

Brandt, Nina; O'Neill, Hayley M; Kleinert, Maximilian

2015-01-01

INTRODUCTION: Members of the interleukin-6 (IL-6) family, IL-6 and ciliary neurotrophic factor (CNTF) have been shown to increase glucose uptake and fatty acid oxidation in skeletal muscle. However, the metabolic effects of another family member, leukemia inhibitory factor (LIF), are not well...

Overview of structure and function of mammalian cilia

DEFF Research Database (Denmark)

Satir, Peter; Christensen, Søren Tvorup

2007-01-01

nonmotile 9 + 0 primary cilia are found on epithelial cells, such as those of the kidney tubule, but also on nonepithelial cells, such as chondrocytes, fibroblasts, and neurons. The ciliary membranes of all cilia contain specific receptors and ion channel proteins that initiate signaling pathways...

Effect of Lactic Acid Lactobacillus Preservative and Moisture Level at Baling on In-situ Digestibility of Crabgrass Hay by Heifers.

Science.gov (United States)

Common crabgrass (Digitaria ciliaris [Retz.] Koel.) is a warm-season annual that offers an advantage over many perennial warm-season grasses because of its greater nutritive value and high palatability. However, little is known about how baling crabgrass hay at high moisture affects ruminal forage ...

Rekombinant humant deoxyribonuklease til andet end cystisk fibrose

DEFF Research Database (Denmark)

Kristensen, Kim

2010-01-01

be associated with increased need for supplemental oxygen. In adults with idiopathic bronchiectasis, treatment with rhDNase leads to more pulmonary exacerbations and a greater decline in pulmonary function tests. There are no controlled studies on rhDNase in primary ciliary dyskinesia or atelectasis....

Information Need and Information Seeking Behaviour

OpenAIRE

Nazan Özenç Uçak

1997-01-01

Information need is one of the cognitive needs of humankind. Information need causes information seeking behaviour and these concepts complement each other. Information need and information seeking behaviour are effected by many factors. It is necessary to know these factors in establishing the effective information centers and services.

Information Need and Information Seeking Behaviour

Directory of Open Access Journals (Sweden)

Nazan Özenç Uçak

1997-12-01

Full Text Available Information need is one of the cognitive needs of humankind. Information need causes information seeking behaviour and these concepts complement each other. Information need and information seeking behaviour are effected by many factors. It is necessary to know these factors in establishing the effective information centers and services.

Information adaptation the interplay between Shannon information and semantic information in cognition

CERN Document Server

Haken, Hermann

2015-01-01

This monograph demonstrates the interplay between Shannon information and semantic information in cognition. It shows that Shannon’s information acts as driving force for the formation of semantic information; and vice versa, namely, that semantic information participates in the formation of Shannonian information. The authors show that in cognition, Shannonian and semantic information are interrelated as two aspects of a cognitive process termed as information adaptation. In the latter the mind/brain adapts to the environment by the deflating and/or inflating of the information conveyed by the environment. In the process of information adaptation, quantitative variations in Shannon’s information entail different meanings while different meanings affect the quantity of information. The book illustrates the above conceptually and mathematically by reference to three cognitive processes: pattern recognition, face learning and the recognition of a moving object.

NJP VOLUME 41 NO 3

African Journals Online (AJOL)

PROF. EZECHUKWU

2013-04-10

Apr 10, 2013 ... unrelated conditions1. Individuals with dextrocardia and situs inversus totalis may have associated congenital heart malformations2, primary ciliary dyskinesia or splenic malformations.3. We describe a case of dextrocardia with situs inversus totalis in a one day old neonate with neonatal sepsis, the.

TCTN2 : a novel tumor marker with oncogenic properties

NARCIS (Netherlands)

Cano-Rodriguez, David; Campagnoli, Susanna; Grandi, Alberto; Parri, Matteo; De Camilli, Elisa; Song, Chaojun; Jin, Boquan; Lacombe, Aurelien; Pierleoni, Andrea; Bombaci, Mauro; Cordiglieri, Chiara; Ruiters, Marcel H. J.; Viale, Giuseppe; Terracciano, Luigi; Sarmientos, Paolo; Abrignani, Sergio; Grandi, Guido; Pileri, Piero; Rots, Marianne G.; Grifantini, Renata

2017-01-01

Tectonic family member 2 (TCTN2) encodes a transmembrane protein that belongs to the tectonic family, which is involved in ciliary functions. Previous studies have demonstrated the role of tectonics in regulating a variety of signaling pathways at the transition zone of cilia. However, the role of

Images in medicine

African Journals Online (AJOL)

ebutamanya

2015-01-29

Jan 29, 2015 ... usually asymptomatic, as the structure and function of vital organs are generally unaffected. However, in 25% of the cases, the syndrome coexists with primary ciliary dyskinesia (Kartagener syndrome), then characterized by chronic sinusitis, bronchiectasis and susceptibility to infections. A random X-ray test ...

Caracterización de producción de etanol a partir de zacate buffel y sus implicaciones ambientales

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Kareen Krizzan Encinas Soto

2016-12-01

Full Text Available En esta investigación se caracterizó el potencial para la obtención de etanol a partir de la planta silvestre Cenchrus ciliaris, también conocida como zacate buffel. A partir de estudios experimentales a nivel laboratorio, se determinaron los rendimientos técnicos y requerimientos energéticos de las etapas de pretratamiento ácido, hidrólisis enzimática y fermentación. Los valores obtenidos fueron comparados con los reportados en la literatura para otros procesos de producción de combustibles. Los resultados del presente trabajo indican que el procesamiento de Cenchrus ciliaris consume la tercera parte de la energía requerida para procesar gasolina por 1 MJ de etanol producido. Asimismo, mediante este proceso es factible la reducción de las emisiones de gases de efecto invernadero por 1 MJ de energía de etanol producido en aproximadamente 40 % su valor actual.

Flagellar Motility of Trypanosoma cruzi Epimastigotes

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G. Ballesteros-Rodea

2012-01-01

Full Text Available The hemoflagellate Trypanosoma cruzi is the causative agent of American trypanosomiasis. Despite the importance of motility in the parasite life cycle, little is known about T. cruzi motility, and there is no quantitative description of its flagellar beating. Using video microscopy and quantitative vectorial analysis of epimastigote trajectories, we find a forward parasite motility defined by tip-to-base symmetrical flagellar beats. This motion is occasionally interrupted by base-to-tip highly asymmetric beats, which represent the ciliary beat of trypanosomatid flagella. The switch between flagellar and ciliary beating facilitates the parasite's reorientation, which produces a large variability of movement and trajectories that results in different distance ranges traveled by the cells. An analysis of the distance, speed, and rotational angle indicates that epimastigote movement is not completely random, and the phenomenon is highly dependent on the parasite behavior and is characterized by directed and tumbling parasite motion as well as their combination, resulting in the alternation of rectilinear and intricate motility paths.

Design considerations of a real-time clinical confocal microscope

Science.gov (United States)

Masters, Barry R.

1991-06-01

A real-time clinical confocal light microscope provides the ophthalmologist with a new tool for the observation of the cornea and the ocular lens. In addition, the ciliary body, the iris, and the sclera can be observed. The real-time light microscopic images have high contrast and resolution. The transverse resolution is about one half micron and the range resolution is one micron. The following observations were made with visible light: corneal epithelial cells, wing cells, basal cells, Bowman's membrane, nerve fibers, basal lamina, fibroblast nuclei, Descemet's membrane, endothelial cells. Observation of the in situ ocular lens showed lens capsule, lens epithelium, lens fibrils, the interior of lens fibrils. The applications of the confocal microscope include: eye banking, laser refractive surgery, observation of wound healing, observation of the iris, the sciera, the ciliary body, the ocular lens, and the intraocular lens. Digital image processing can produce three-dimensional reconstructions of the cornea and the ocular lens.

VAMP7 modulates ciliary biogenesis in kidney cells.

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Christina M Szalinski

Full Text Available Epithelial cells elaborate specialized domains that have distinct protein and lipid compositions, including the apical and basolateral surfaces and primary cilia. Maintaining the identity of these domains is required for proper cell function, and requires the efficient and selective SNARE-mediated fusion of vesicles containing newly synthesized and recycling proteins with the proper target membrane. Multiple pathways exist to deliver newly synthesized proteins to the apical surface of kidney cells, and the post-Golgi SNAREs, or VAMPs, involved in these distinct pathways have not been identified. VAMP7 has been implicated in apical protein delivery in other cell types, and we hypothesized that this SNARE would have differential effects on the trafficking of apical proteins known to take distinct routes to the apical surface in kidney cells. VAMP7 expressed in polarized Madin Darby canine kidney cells colocalized primarily with LAMP2-positive compartments, and siRNA-mediated knockdown modulated lysosome size, consistent with the known function of VAMP7 in lysosomal delivery. Surprisingly, VAMP7 knockdown had no effect on apical delivery of numerous cargoes tested, but did decrease the length and frequency of primary cilia. Additionally, VAMP7 knockdown disrupted cystogenesis in cells grown in a three-dimensional basement membrane matrix. The effects of VAMP7 depletion on ciliogenesis and cystogenesis are not directly linked to the disruption of lysosomal function, as cilia lengths and cyst morphology were unaffected in an MDCK lysosomal storage disorder model. Together, our data suggest that VAMP7 plays an essential role in ciliogenesis and lumen formation. To our knowledge, this is the first study implicating an R-SNARE in ciliogenesis and cystogenesis.

Artificial cilia : a physical model for ciliary propulsion

OpenAIRE

Babataheri , Avin

2009-01-01

Most microorganisms use cilia or flagella as a means of propulsion. These low Reynolds number swimming mechanisms have been studied theoretically and experimentally on living organisms. However, so far very few physical experimental models have been realised. We describe here the fabrication of microscopic artificial cilia, actuated by a magnetic field. These artificial cilia share with real cilia a large aspect ratio, great flexibility, and the actuation by a magnetic torque distributed alon...

Information,Informal finance,and SME financing

Institute of Scientific and Technical Information of China (English)

LIN Justin Yifu; SUN Xifang

2006-01-01

Informal finance exists extensively and has been playing an important role in small-and medium-sized enterprise (SME) financing in developing economies,This paper tries to rationalize the extensiveness of informal finance.SME financing suffers more serious information asymmetry to the extent that most SMEs are more opaque and can only provide less collateral.Informal lenders have an advantage over formal financial institutions in collecting "soft information" about SME borrowers.This paper establishes a model including formal and informal lenders and high-and low-risk borrowers with or without sufficient collateral and shows that the credit market in which informal finance is eliminated will allocate funds in some inefficient way,and the efficiency of allocating credit funds can be improved once informal finance is allowed to coexist with formal finance.

Informed consent: information or knowledge?

Science.gov (United States)

Berger, Ken

2003-01-01

A fiduciary relationship should be nurtured between patient and physician. This requires effective communication throughout all aspects of care - especially pertaining to treatment decisions. In the context of illness as experienced by the patient a unique set of circumstances is presented. However, communication in an illness context is fraught with problems. The patient is vulnerable and the situation may be overwhelming. Voluminous amounts of information are available to patients from a host of health care providers, family members, support groups, advocacy centers, books, journals, and the internet. Often conflicting and confusion, frequently complex, this information may be of greater burden than benefit. Some information is of high validity and reliability while other information is of dubious reliability. The emotional freight of bad news may further inhibit understanding. An overload of information may pose an obstacle in decision-making. To facilitate the transformation of information into knowledge, the health care provider must act on some occasions as a filter, on other occasions as a conduit, and on still other occasions simply as a reservoir. The evolution of patient rights to receive or refuse treatment, the right to know or not to know calls for a change in processing of overwhelming information in our modem era. In this paper we will discuss the difference between information and knowledge. How can health care providers ensure they have given their patients all necessary and sufficient information to make an autonomous decision? How can they facilitate the transformation of information into knowledge? The effect of knowledge to consent allows a more focused, relevant and modern approach to choice in health care.

PDGFR alpha signaling in the primary cilium regulates NHE1-dependent fibroblast migration via coordinated differential activity of MEK1/2-ERK1/2-p90(RSK) and AKT signaling pathways

DEFF Research Database (Denmark)

Clement, Ditte L.; Mally, Sabine; Stock, Christian

2013-01-01

In fibroblasts, platelet-derived growth factor receptor alpha (PDGFR alpha) is upregulated during growth arrest and compartmentalized to the primary cilium. PDGF-AA mediated activation of the dimerized ciliary receptor produces a phosphorylation cascade through the PI3K-AKT and MEK1/2-ERK1/2 path...

Oxygen dynamics and porewater transport in sediments inhabited by the invasive polychaete Marenzelleria viridis

DEFF Research Database (Denmark)

Jovanovic, Zeljko; Larsen, Morten; Quintana, Cintia Organo

2014-01-01

(rhodamine WT and brilliant blue), we investigated the relationship between irrigation and O2 dynamics in burrows of M. viridis. The investigated animals shifted between 2 modes of ventilation: ciliary pumping for 77% of the time and muscular pumping for 23% of the time. On average, muscular pumping...

Water permeability of Na+-K+-2Cl- cotransporters in mammalian epithelial cells

DEFF Research Database (Denmark)

Hamann, Steffen; Herrera-Perez, José Jaime; Bundgaard, Magnus

2005-01-01

Water transport properties of the Na+-K+-2Cl- cotransporter (NKCC) were studied in cultures of pigmented epithelial cells (PE) from the ciliary body of the eye. Here, the membrane that faces upwards contains NKCCs and can be subjected to rapid changes in bathing solution composition and osmolarity...

Seed salt-soluble protein expression as marker of local Medicago ...

African Journals Online (AJOL)

Eleven patterns of M. ciliaris populations with two moderate sensitive ecotypes to NaCl, two reference genotypes and seven prospecting populations near and far from a strongly salted area (Sebkha of Oran) were investigated by one dimensional electrophoresis SDS-PAGE. The results show that the proteins profiles were ...

Fine structure of the creeping larva of Loxosomella murmanica: additional evidence for a clade of Kamptozoa (Entoprocta) and Mollusca

DEFF Research Database (Denmark)

Haszprunar, Gerhard; Wanninger, Andreas Wilhelm Georg

2008-01-01

. The prototroch consists of two ciliary rings; a downstream collecting system is not present. Although there is a one-way gut with a lumen throughout, the larva obviously does not feed. A single pair of protonephridia is present. The foot sole shares distinct similarities with basic molluscs, particularly...

Association of vitamin D receptor gene start codon (Fok1 polymorphism with high myopia

Directory of Open Access Journals (Sweden)

Sandhya Annamaneni

2011-01-01

Conclusion : The results suggest that VDR gene might not be playing a direct role in the development of myopia, but might contribute indirectly to the risk conferred by mechanical stress factors or growth/development related factors through its role in calcium homeostasis and regulation of ciliary muscle function.

Ploidy determination of buffel grass accessions in the USDA National Plant Germplasm System collection by flow cytometry

Science.gov (United States)

Buffelgrass [Pennisetum ciliare (L.) Link syn. Cenchrus ciliaris L.] is an important forage and range grass in many of the semi-arid tropical and subtropical regions of the world. The species reproduces primarily by apomixis but it is highly diverse because a wide array of different apomictic ecoty...

Information management - Assessing the demand for information

Science.gov (United States)

Rogers, William H.

1991-01-01

Information demand is defined in terms of both information content (what information) and form (when, how, and where it is needed). Providing the information richness required for flight crews to be informed without overwhelming their information processing capabilities will require a great deal of automated intelligence. It is seen that the essence of this intelligence is comprehending and capturing the demand for information.

Total mercury and methylmercury accumulation in wild plants grown at wastelands composed of mine tailings: Insights into potential candidates for phytoremediation.

Science.gov (United States)

Qian, Xiaoli; Wu, Yonggui; Zhou, Hongyun; Xu, Xiaohang; Xu, Zhidong; Shang, Lihai; Qiu, Guangle

2018-08-01

Total mercury (THg) and methylmercury (MMHg) were investigated in 259 wild plants belonging to 49 species in 29 families that grew in heavily Hg-contaminated wastelands composed of cinnabar ore mine tailings (calcines) in the Wanshan region, southwestern China, the world's third largest Hg mining district. The bioconcentration factors (BCFs) of THg and MMHg from soil to roots ([THg] root /[THg] soil , [MMHg] root /[MMHg] soil ) were evaluated. The results showed that THg and MMHg in both plants and soils varied widely, with ranges of 0.076-140 μg/g THg and 0.19-87 ng/g MMHg in roots, 0.19-106 μg/g THg and 0.06-31 ng/g MMHg in shoots, and 0.74-1440 μg/g THg and 0.41-820 ng/g MMHg in soil. Among all investigated species, Arthraxon hispidus, Eremochloa ciliaris, Clerodendrum bunge, and Ixeris sonchifolia had significantly elevated concentrations of THg in shoots and/or roots that reached 100 μg/g, whereas Chenopodium glaucum, Corydalisedulis maxim, and Rumex acetosa contained low values below 0.5 μg/g. In addition to the high THg concentrations, the fern E. ciliaris also showed high BCF values for both THg and MMHg exceeding 1.0, suggesting its capability to extract Hg from soils. Considering its dominance and the tolerance identified in the present study, E. ciliaris is suggested to be a practical candidate for phytoextraction, whereas A. hispidus is identified as a potential candidate for phytostabilization of Hg mining-contaminated soils. Copyright © 2018 Elsevier Ltd. All rights reserved.

Ultrasound biomicroscopy in patients with unilateral pseudoexfoliation

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Erkan Ünsal

2015-08-01

Full Text Available AIM: To compare the anterior segment morphology evaluated using ultrasound biomicroscopy (UBM in patients with clinical pseudoexfoliation syndrome (XFS in one eye and no clinical XFS in the fellow eye.METHODS: Thirty patients with unilateral XFS were included in the study. All patients underwent evaluation of their anterior segment using UBM with and without dilatation with 1% cyclopentolate. The anterior chamber depth (ACD, lens thickness (LT, anterior chamber angle (ACA, ciliary body thickness (CBT, scleral thickness (ST, trabeculae -ciliary processes distance (T-CPD, and iris-ciliary processes distance (I-CPD were measured using UBM scans. All results between the eyes with clinical XFS and their fellow eyes without clinical XFS were then compared.RESULTS: Before dilatation the eyes with XFS (4.350±0.531 mm were found to have a significantly thicker lens (P=0.002 than the eyes without XFS (4.238±0.540 mm. In addition after dilatation, the eyes with XFS (4.310±0.500 mm were found to have a significantly thicker lens than the eyes without XFS (4.160±0.480 mm (P=0.019. The average ACD, for the group with XFS, comparing pre-dilatation (2.616±0.349 mm and post-dilatation measurements (2.714±0.413 was found to be statistically increased (P=0.014. The average ACD, comparing pre-dilatation to post-dilatation measurements in patients without XFS (2.680±0.360, (2.720±0.500 was found to be statistically unchanged (P=0.450.DISCUSSION: Crystalline lenses tended to be thicker in the eyes with clinical pseudoexfoliation than their fellow eyes without pseudoexfoliation.

Evaluation of diode endoscopic cyclophotocoagulation in bovine cadaver eyes.

Science.gov (United States)

Harrington, Jay T; McMullen, Richard J; Cullen, John M; Gilger, Brian C

2012-09-01

To evaluate the anterior chamber approach and energy levels for endoscopic cyclophotocoagulation (ECPC) and assess ECPC-induced tissue damage in phakic eyes of bovine cadavers. 12 bovine cadaver eyes. Angle of reach was measured in 6 eyes following placement of a curved endoscopic probe through multiple corneal incisions. In another 6 eyes, each ocular quadrant underwent ECPC at 1 of 3 energy levels (0.75, 0.90, and 1.05 J) or remained untreated. Visible effects on tissues (whitening and contraction of ciliary processes) were scored (scale of 0 [no effects] to 6 [severe effects]), and severity and extent of histologic damage to the pigmented and nonpigmented ciliary epithelium and fibromuscular stroma were each scored (scale of 0 [no effect] to 3 [severe effect]) and summed for each quadrant. Overall mean scores for 6 quadrants/treatment were calculated. Mean ± SD combined angle of reach was 148 ± 24° (range, 123 ± 23° [ventromedial] to 174 ± 11° [dorsolateral]). At the 0.75-, 0.90-, and 1.05-J levels, mean visible tissue effect scores were 3.12 ± 0.47, 3.86 ± 0.35, and 4.68 ± 0.58, respectively; mean histologic damage scores were 4.79 ± 1.38 (mild damage), 6.82 ± 1.47 (moderate damage), and 9.37 ± 1.42 (severe damage), respectively. Occasional popping noises (venting of vaporized interstitial water) were heard at the 1.05-J level. Multiple incisions were necessary to facilitate 360° ECPC treatment in bovine eyes. For ECPC in vivo, the 0.75- and 0.90-J energy levels had the potential to effectively treat the ciliary epithelium.

De novo formation of left-right asymmetry by posterior tilt of nodal cilia.

Directory of Open Access Journals (Sweden)

Shigenori Nonaka

2005-08-01

Full Text Available In the developing mouse embryo, leftward fluid flow on the ventral side of the node determines left-right (L-R asymmetry. However, the mechanism by which the rotational movement of node cilia can generate a unidirectional flow remains hypothetical. Here we have addressed this question by motion and morphological analyses of the node cilia and by fluid dynamic model experiments. We found that the cilia stand, not perpendicular to the node surface, but tilted posteriorly. We further confirmed that such posterior tilt can produce leftward flow in model experiments. These results strongly suggest that L-R asymmetry is not the descendant of pre-existing L-R asymmetry within each cell but is generated de novo by combining three sources of spatial information: antero-posterior and dorso-ventral axes, and the chirality of ciliary movement.

Cotransport of water by the Na+-K+-2Cl(-) cotransporter NKCC1 in mammalian epithelial cells

DEFF Research Database (Denmark)

Hamann, Steffen; Herrera-Perez, José J; Zeuthen, Thomas

2010-01-01

Water transport by the Na+-K+-2Cl(-) cotransporter (NKCC1) was studied in confluent cultures of pigmented epithelial (PE) cells from the ciliary body of the fetal human eye. Interdependence among water, Na+ and Cl(-) fluxes mediated by NKCC1 was inferred from changes in cell water volume, monitored...

Optical techniques in pulmonary medicine. SPIE photonics West.

Science.gov (United States)

Suter, Melissa J; Lam, Stephen; Brenner, Matthew

2012-04-01

There is ongoing interest in the emerging field of pulmonary photonic-based diagnostics. Potential clinical need areas that are being actively investigated at this time include airway and peripheral lung cancer diagnostics, pulmonary parenchymal and interstitial disorders, alveolar structure function, inhalation injury, ciliary function analysis, asthma and obstructive lung diseases.

Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog.

Science.gov (United States)

Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

2011-11-01

A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener's syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

Information asymmetries, information externalities, oil companies strategies and oil exploration information efficiency

International Nuclear Information System (INIS)

Nyouki, E.

1998-07-01

Both for economics (in general) and energy economics matters, it is important to reach oil exploration efficiency. To achieve this aim, a pragmatic approach is to use the concept of information efficiency which means that the different tracts have to be drilled in the decreasing order of estimated profitabilities, estimations being made on the basis of the best (in the sense of reliability) available information. What does 'best available information' mean? It corresponds either to the information held by the most experienced oil companies (due to the existence of information asymmetries to the profit of these companies), or to information revealed by the drilling and which allows to revise probabilities of success on neighboring tracts with similar geological features (due to the existence of information externalities). In consideration of these information asymmetries and externalities, we will say that exploration is information efficient when. -- on the one hand, initial exploration choices are directed by the most experienced companies, - and, on the other hand, during the drilling phase, in the face of the information externality, companies adopt a sequential drilling, i.e. excluding both over-investment and strategic under-investment. The topic we deal with in this thesis is then to know if oil companies, when they are put in normal competition conditions, are likely to make emerge a state of information efficiency in exploration, the analysis being conducted theoretically and empirically. (author)

Information in organisations: directions for information management

Directory of Open Access Journals (Sweden)

Joyce Kirk

1999-01-01

Full Text Available The work of managers in small and medium-sized enterprises is very information-intensive and the environment in which it is done is very information rich. But are managers able to exploit the wealth of information which surrounds them? And how can information be managed in organisations so that its potential for improving business performance and enhancing the competitiveness of these enterprises can be realised? Answers to these questions lie in clarifying the context of the practice of information management by exploring aspects of organisations and managerial work and in exploring the nature of information at the level of the organisation and the individual manager. From these answers it is possible to suggest some guidelines for managing the integration of business strategy and information, the adoption of a broadly-based definition of information and the development of information capabilities.

Informal information for web-based engineering catalogues

Science.gov (United States)

Allen, Richard D.; Culley, Stephen J.; Hicks, Ben J.

2001-10-01

Success is highly dependent on the ability of a company to efficiently produce optimal designs. In order to achieve this companies must minimize time to market and possess the ability to make fully informed decisions at the early phase of the design process. Such decisions may include the choice of component and suppliers, as well as cost and maintenance considerations. Computer modeling and electronic catalogues are becoming the preferred medium for the selection and design of mechanical components. In utilizing these techniques, the designer demands the capability to identify, evaluate and select mechanical components both quantitatively and qualitatively. Quantitative decisions generally encompass performance data included in the formal catalogue representation. It is in the area of qualitative decisions that the use of what the authors call 'Informal Information' is of crucial importance. Thus, 'Informal Information' must often be incorporated into the selection process and selection systems. This would enable more informed decisions to be made quicker, without the need for information retrieval via discussion with colleagues in the design environment. This paper provides an overview of the use of electronic information in the design of mechanical systems, including a discussion of limitations of current technology. The importance of Informal Information is discussed and the requirements for association with web based electronic catalogues are developed. This system is based on a flexible XML schema and enables the storage, classification and recall of Informal Information packets. Furthermore, a strategy for the inclusion of Informal Information is proposed, and an example case is used to illustrate the benefits.

Information Impact: Journal of Information and Knowledge ...

African Journals Online (AJOL)

Information Impact: Journal of Information and Knowledge Management: Advanced Search. Journal Home > Information Impact: Journal of Information and Knowledge Management: Advanced Search. Log in or Register to get access to full text downloads.

Information in Our World: Conceptions of Information and Problems of Method in Information Science

Science.gov (United States)

Ma, Lai

2012-01-01

Many concepts of information have been proposed and discussed in library and information science. These concepts of information can be broadly categorized as empirical and situational information. Unlike nomenclatures in many sciences, however, the concept of information in library and information science does not bear a generally accepted…

Structure and development of the saccular sensory epithelium in ...

African Journals Online (AJOL)

Structure and development of the saccular sensory epithelium in relation to otolith growth in the perch Perca fluviatilis (Telostei) ... Electron microscopy indicated: 1) The apical surface of each hair cell is covered with a ciliary bundle which varies in length in different epithelial regions. Each bundle is formed from a long ...

Situs inversus totalis associated with subaortic stenosis, restrictive ventricular septal defect, and tricuspid dysplasia in an adult dog

OpenAIRE

Piantedosi, Diego; Cortese, Laura; Meomartino, Leonardo; Di Loria, Antonio; Ciaramella, Paolo

2011-01-01

A rare association between situs inversus totalis (SIT), restrictive ventricular septal defect, severe subaortic stenosis, and tricuspid dysplasia was observed in an adult mixed-breed dog. Primary ciliary dyskinesia and Kartagener’s syndrome were excluded. After 15 mo the dog died suddenly. The association between SIT and congenital heart diseases is discussed.

Soil erosion studies in buffelgrass pastures

Science.gov (United States)

Diego Valdez-Zamudio; D. Phillip Guertin

2000-01-01

The introduction of exotic grasses in native rangelands to increase the production of forage has been a good alternative for the cattle industry in North America. Different studies have demonstrated that buffelgrass (Cenchrus ciliaris L.), a plant introduced from Africa, increases the annual green forage production approximately three times in comparison to production...

Information Impact: Journal of Information and Knowledge ...

African Journals Online (AJOL)

Information Impact: Journal of Information and Knowledge Management: Site Map. Journal Home > About the Journal > Information Impact: Journal of Information and Knowledge Management: Site Map. Log in or Register to get access to full text downloads.

Role of Information Anxiety and Information Load on Processing of Prescription Drug Information Leaflets.

Science.gov (United States)

Bapat, Shweta S; Patel, Harshali K; Sansgiry, Sujit S

2017-10-16

In this study, we evaluate the role of information anxiety and information load on the intention to read information from prescription drug information leaflets (PILs). These PILs were developed based on the principals of information load and consumer information processing. This was an experimental prospective repeated measures study conducted in the United States where 360 (62% response rate) university students (>18 years old) participated. Participants were presented with a scenario followed by exposure to the three drug product information sources used to operationalize information load. The three sources were: (i) current practice; (ii) pre-existing one-page text only; and (iii) interventional one-page prototype PILs designed for the study. Information anxiety was measured as anxiety experienced by the individual when encountering information. The outcome variable of intention to read PILs was defined as the likelihood that the patient will read the information provided in the leaflets. A survey questionnaire was used to capture the data and the objectives were analyzed by performing a repeated measures MANOVA using SAS version 9.3. When compared to current practice and one-page text only leaflets, one-page PILs had significantly lower scores on information anxiety ( p information load ( p Information anxiety and information load significantly impacted intention to read ( p < 0.001). Newly developed PILs increased patient's intention to read and can help in improving the counseling services provided by pharmacists.

Information Architecture the Design of Digital Information Spaces

CERN Document Server

Ding, Wei

2009-01-01

Information Architecture is about organizing and simplifying information, designing and integrating information spaces/systems, and creating ways for people to find and interact with information content. Its goal is to help people understand and manage information and make right decisions accordingly. In the ever-changing social, organizational and technological contexts, Information Architects not only design individual information spaces (e.g., individual websites, software applications, and mobile devices), but also tackle strategic aggregation and integration of multiple information spaces

Roles of libraries in information society | Awojobi | Information ...

African Journals Online (AJOL)

Through the revi ew of relevant literature, the paper examined the roles of libraries in information society. The information society is characterised by proliferation of information through the use of Information and Communication Technology (ICT). One major principle of an information society is accessibility to information ...

Information Warfare, Threats and Information Security

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Dmitriy Nikolaevich Bespalov

2014-01-01

Full Text Available The article presents the opposite, but dependent on each other's reality - Revolutionary War information,information security goals and objectives of their study within the scheme "challenge-response", methodological and analytical support, the role of elites and the information society in promoting information security. One of the features of contemporaneityis the global spread of ICT, combined with poor governance and other difficulties in the construction of innovation infrastructures that are based on them in some countries. This leads to the reproduction of threats, primarily related to the ability to use ICT for purposes that are inconsistent with the objectives of maintaining international peace and security, compliance with the principles of non-use of force, non-interference in the internal affairs of states, etc. In this regard, include such terms as "a threat of information warfare", "information terrorism" and so forth. Information warfare, which stay in the policy declared the struggle for existence, and relationships are defined in terms of "friend-enemy", "ours-foreign". Superiority over the opponent or "capture of its territory" is the aim of political activity. And information security, serving activities similar process of political control, including a set of components, is a technology until their humanitarian. From the context and the decision itself is the ratio of the achieved results of information and political influence to the target - a positive image of Russia. Bringing its policy in line with the demands of a healthy public opinion provides conductivity of theauthorities initiatives in the country and increases the legitimacy of the Russian Federation actions in the world.

Information Sensitive Consumers and Market Information.

Science.gov (United States)

Price, Linda L.; And Others

1987-01-01

Past research on consumer information has emphasized the effects of informed consumers of the provision of goods by sellers. This paper examines the effects of informed consumers on other consumers' product choices. These are demand-side effects. Directions for research are outlined. Author/CH)

Information Skills for an Information Age?

Science.gov (United States)

Gawith, Gwen

1986-01-01

Although information skills are the most basic of skills, the tendency is to teach strategies related to educational projects, erroneously assuming that these "information skills" are applicable to everyday decision-making. Educated imaginations are needed for today's variety of lifelong creative information situations. (17 references)…

Information operation/information warfare modeling and simulation

OpenAIRE

Buettner, Raymond

2000-01-01

Information Operations have always been a part of warfare. However, this aspect of warfare is having ever-greater importance as forces rely more and more on information as an enabler. Modern information systems make possible very rapid creation, distribution, and utilization of information. These same systems have vulnerabilities that can be exploited by enemy forces. Information force-on-force is important and complex. New tools and procedures are needed for this warfare arena. As these t...

Structural defects in cilia of the choroid plexus, subfornical organ and ventricular ependyma are associated with ventriculomegaly

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Swiderski Ruth E

2012-10-01

Full Text Available Abstract Background Hydrocephalus is a heterogeneous disorder with multiple etiologies that are not yet fully understood. Animal models have implicated dysfunctional cilia of the ependyma and choroid plexus in the development of the disorder. In this report, we sought to determine the origin of the ventriculomegaly in four Bardet Biedl syndrome (BBS mutant mouse strains as models of a ciliopathy. Methods Evans Blue dye was injected into the lateral ventricle of wild- type and BBS mutant mice to determine whether obstruction of intra- or extra-ventricular CSF flow contributed to ventriculomegaly. Transmission electron microscopy (TEM was used to examine the ultrastructure of the choroid plexus, subfornical organ (SFO, subcommisural organ (SCO, and ventricular ependyma to evaluate their ultrastructure and the morphology of their primary and motile cilia. Results and discussion No obstruction of intra- or extra-ventricular CSF flow was observed, implying a communicating form of hydrocephalus in BBS mutant mice. TEM analyses of the mutants showed no evidence of choroidal papillomas or breakdown of the blood:CSF barrier. In contrast, structural defects were observed in a subpopulation of cilia lining the choroid plexus, SFO, and ventricular ependyma. These included disruptions of the microtubular structure of the axoneme and the presence of electron-dense vesicular-like material along the ciliary shaft and at the tips of cilia. Conclusions Abnormalities in cilia structure and function have the potential to influence ciliary intraflagellar transport (IFT, cilia maintenance, protein trafficking, and regulation of CSF production. Ciliary structural defects are the only consistent pathological features associated with CSF-related structures in BBS mutant mice. These defects are observed from an early age, and may contribute to the underlying pathophysiology of ventriculomegaly.

Mutations in CEP78 Cause Cone-Rod Dystrophy and Hearing Loss Associated with Primary-Cilia Defects.

Science.gov (United States)

Nikopoulos, Konstantinos; Farinelli, Pietro; Giangreco, Basilio; Tsika, Chrysanthi; Royer-Bertrand, Beryl; Mbefo, Martial K; Bedoni, Nicola; Kjellström, Ulrika; El Zaoui, Ikram; Di Gioia, Silvio Alessandro; Balzano, Sara; Cisarova, Katarina; Messina, Andrea; Decembrini, Sarah; Plainis, Sotiris; Blazaki, Styliani V; Khan, Muhammad Imran; Micheal, Shazia; Boldt, Karsten; Ueffing, Marius; Moulin, Alexandre P; Cremers, Frans P M; Roepman, Ronald; Arsenijevic, Yvan; Tsilimbaris, Miltiadis K; Andréasson, Sten; Rivolta, Carlo

2016-09-01

Cone-rod degeneration (CRD) belongs to the disease spectrum of retinal degenerations, a group of hereditary disorders characterized by an extreme clinical and genetic heterogeneity. It mainly differentiates from other retinal dystrophies, and in particular from the more frequent disease retinitis pigmentosa, because cone photoreceptors degenerate at a higher rate than rod photoreceptors, causing severe deficiency of central vision. After exome analysis of a cohort of individuals with CRD, we identified biallelic mutations in the orphan gene CEP78 in three subjects from two families: one from Greece and another from Sweden. The Greek subject, from the island of Crete, was homozygous for the c.499+1G>T (IVS3+1G>T) mutation in intron 3. The Swedish subjects, two siblings, were compound heterozygotes for the nearby mutation c.499+5G>A (IVS3+5G>A) and for the frameshift-causing variant c.633delC (p.Trp212Glyfs(∗)18). In addition to CRD, these three individuals had hearing loss or hearing deficit. Immunostaining highlighted the presence of CEP78 in the inner segments of retinal photoreceptors, predominantly of cones, and at the base of the primary cilium of fibroblasts. Interaction studies also showed that CEP78 binds to FAM161A, another ciliary protein associated with retinal degeneration. Finally, analysis of skin fibroblasts derived from affected individuals revealed abnormal ciliary morphology, as compared to that of control cells. Altogether, our data strongly suggest that mutations in CEP78 cause a previously undescribed clinical entity of a ciliary nature characterized by blindness and deafness but clearly distinct from Usher syndrome, a condition for which visual impairment is due to retinitis pigmentosa. Copyright © 2016 American Society of Human Genetics. Published by Elsevier Inc. All rights reserved.

The Role of Quinine-Responsive Taste Receptor Family 2 in Airway Immune Defense and Chronic Rhinosinusitis

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Alan D. Workman

2018-03-01

Full Text Available BackgroundBitter (T2R and sweet (T1R taste receptors in the airway are important in innate immune defense, and variations in taste receptor functionality in one T2R (T2R38 correlate with disease status and disease severity in chronic rhinosinusitis (CRS. Quinine is a bitter compound that is an agonist for several T2Rs also expressed on sinonasal cells, but not for T2R38. Because of this property, quinine may stimulate innate immune defense mechanisms in the airway, and functional differences in quinine perception may be reflective of disease status in CRS.MethodsDemographic and taste intensity data were collected prospectively from CRS patients and non-CRS control subjects. Sinonasal tissue from patients undergoing rhinologic surgery was also collected and grown at an air–liquid interface (ALI. Nitric oxide (NO production and dynamic regulation of ciliary beat frequency in response to quinine stimulation were assessed in vitro.ResultsQuinine reliably increased ciliary beat frequency and NO production in ALI cultures in a manner consistent with T2R activation (p < 0.01. Quinine taste intensity rating was performed in 328 CRS patients and 287 control subjects demonstrating that CRS with nasal polyps (CRSwNP patients rated quinine as significantly less intense than did control subjects.ConclusionQuinine stimulates airway innate immune defenses by increasing ciliary beat frequency and stimulating NO production in a manner fitting with T2R activation. Patient variability in quinine sensitivity is observed in taste intensity ratings, and gustatory quinine “insensitivity” is associated with CRSwNP status. Thus, taste tests for quinine may be a biomarker for CRSwNP, and topical quinine has therapeutic potential as a stimulant of innate defenses.

Cis-regulatory control of the nuclear receptor Coup-TF gene in the sea urchin Paracentrotus lividus embryo.

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Lamprini G Kalampoki

Full Text Available Coup-TF, an orphan member of the nuclear receptor super family, has a fundamental role in the development of metazoan embryos. The study of the gene's regulatory circuit in the sea urchin embryo will facilitate the placement of this transcription factor in the well-studied embryonic Gene Regulatory Network (GRN. The Paracentrotus lividus Coup-TF gene (PlCoup-TF is expressed throughout embryonic development preferentially in the oral ectoderm of the gastrula and the ciliary band of the pluteus stage. Two overlapping λ genomic clones, containing three exons and upstream sequences of PlCoup-TF, were isolated from a genomic library. The transcription initiation site was determined and 5' deletions and individual segments of a 1930 bp upstream region were placed ahead of a GFP reporter cassette and injected into fertilized P.lividus eggs. Module a (-532 to -232, was necessary and sufficient to confer ciliary band expression to the reporter. Comparison of P.lividus and Strongylocentrotus purpuratus upstream Coup-TF sequences, revealed considerable conservation, but none within module a. 5' and internal deletions into module a, defined a smaller region that confers ciliary band specific expression. Putative regulatory cis-acting elements (RE1, RE2 and RE3 within module a, were specifically bound by proteins in sea urchin embryonic nuclear extracts. Site-specific mutagenesis of these elements resulted in loss of reporter activity (RE1 or ectopic expression (RE2, RE3. It is proposed that sea urchin transcription factors, which bind these three regulatory sites, are necessary for spatial and quantitative regulation of the PlCoup-TF gene at pluteus stage sea urchin embryos. These findings lead to the future identification of these factors and to the hierarchical positioning of PlCoup-TF within the embryonic GRN.

Missense mutations in the WD40 domain of AHI1 cause non-syndromic retinitis pigmentosa.

Science.gov (United States)

Nguyen, Thanh-Minh T; Hull, Sarah; Roepman, Ronald; van den Born, L Ingeborgh; Oud, Machteld M; de Vrieze, Erik; Hetterschijt, Lisette; Letteboer, Stef J F; van Beersum, Sylvia E C; Blokland, Ellen A; Yntema, Helger G; Cremers, Frans P M; van der Zwaag, Paul A; Arno, Gavin; van Wijk, Erwin; Webster, Andrew R; Haer-Wigman, Lonneke

2017-09-01

Recent findings suggesting that Abelson helper integration site 1 ( AHI1 ) is involved in non-syndromic retinal disease have been debated, as the functional significance of identified missense variants was uncertain. We assessed whether AHI1 variants cause non-syndromic retinitis pigmentosa (RP). Exome sequencing was performed in three probands with RP. The effects of the identified missense variants in AHI1 were predicted by three-dimensional structure homology modelling. Ciliary parameters were evaluated in patient's fibroblasts, and recombinant mutant proteins were expressed in ciliated retinal pigmented epithelium cells. In the three patients with RP, three sets of compound heterozygous variants were detected in AHI1 (c.2174G>A; p.Trp725* and c.2258A>T; p.Asp753Val, c.660delC; p.Ser221Glnfs*10 and c.2090C>T; p.Pro697Leu, c.2087A>G; p.His696Arg and c.2429C>T; p.Pro810Leu). All four missense variants were present in the conserved WD40 domain of Jouberin, the ciliary protein encoded by AHI1 , with variable predicted implications for the domain structure. No significant changes in the percentage of ciliated cells, nor in cilium length or intraflagellar transport were detected. However, expression of mutant recombinant Jouberin in ciliated cells showed a significantly decreased enrichment at the ciliary base. This report confirms that mutations in AHI1 can underlie autosomal recessive RP. Moreover, it structurally and functionally validates the effect of the RP-associated AHI1 variants on protein function, thus proposing a new genotype-phenotype correlation for AHI1 mutation associated retinal ciliopathies. © Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

A homozygous nonsense CEP250 mutation combined with a heterozygous nonsense C2orf71 mutation is associated with atypical Usher syndrome.

Science.gov (United States)

Khateb, Samer; Zelinger, Lina; Mizrahi-Meissonnier, Liliana; Ayuso, Carmen; Koenekoop, Robert K; Laxer, Uri; Gross, Menachem; Banin, Eyal; Sharon, Dror

2014-07-01

Usher syndrome (USH) is a heterogeneous group of inherited retinitis pigmentosa (RP) and sensorineural hearing loss (SNHL) caused by mutations in at least 12 genes. Our aim is to identify additional USH-related genes. Clinical examination included visual acuity test, funduscopy and electroretinography. Genetic analysis included homozygosity mapping and whole exome sequencing (WES). A combination of homozygosity mapping and WES in a large consanguineous family of Iranian Jewish origin revealed nonsense mutations in two ciliary genes: c.3289C>T (p.Q1097*) in C2orf71 and c.3463C>T (p.R1155*) in centrosome-associated protein CEP250 (C-Nap1). The latter has not been associated with any inherited disease and the c.3463C>T mutation was absent in control chromosomes. Patients who were double homozygotes had SNHL accompanied by early-onset and severe RP, while patients who were homozygous for the CEP250 mutation and carried a single mutant C2orf71 allele had SNHL with mild retinal degeneration. No ciliary structural abnormalities in the respiratory system were evident by electron microscopy analysis. CEP250 expression analysis of the mutant allele revealed the generation of a truncated protein lacking the NEK2-phosphorylation region. A homozygous nonsense CEP250 mutation, in combination with a heterozygous C2orf71 nonsense mutation, causes an atypical form of USH, characterised by early-onset SNHL and a relatively mild RP. The severe retinal involvement in the double homozygotes indicates an additive effect caused by nonsense mutations in genes encoding ciliary proteins. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

Disorders in melanopsin effect of pupil constriction as a risk factor causing eye diseases

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V.A. Kaptsov

2017-03-01

Full Text Available Risks of eye damage and eyesight deterioration to a great extent depend on how efficient a biomechanical eye system is under energy-saving lighting conditions. The system's efficiency is determined by its adequacy in managing pupils and ciliary muscle. We analyzed mathematical models describing changes in pupil's diameter which were determined by light-technical parameters of illumination environment (luminance level and brightness. We highlighted the importance of ganglionic cells and the role they play in managing pupil's diameter (miosis when they are exposed to blue light within 480 nm spectrum. Basing on the assessment of a pupil's constriction under exposure to various light stimuli (blue, red, and green ones we worked out a melanopsin effect concept of a pupil's retention at miosis and showed that it could be a diagnostic sign of some diseases (age-related direct retinopathy, pancreatic diabetes under exposure to a blue light impulse with a certain wave length. Under exposure to blue light within 480 nm spectrum ganglionic cells form a managing signal for a sphincter muscle of a pupil and ciliary muscle which provides accommodation (as per Helmholtz and regulates aqueous humor flow in ciliary channel. All modern energy-saving light sources have a low energy level at wave length equal to 480 nm due to gap in their spectrum in comparison with sunlight spectrum with the same light temperature and luminance level. Inadequate management of pupil's diameter under artificial lighting conditions leads to melanopsin effect disorders and causes disharmony in managing aqueous humor outflow. All the above-stated factors under long-term visual load cause eye diseases risks in modern illumination environment. We detected that contemporary mathematic models describing pupil's diameter fluctuations needed to be refined allowing for new knowledge on functional peculiarities of retina cells and energy-saving light sources spectrum.

O muco traqueobrônquico humano mantido em temperatura ambiente e suas propriedades físico-químicas Physicochemical properties of human tracheobronchial sputum maintained at room temperature

Directory of Open Access Journals (Sweden)

Renata Claudia Zanchet

2007-02-01

Full Text Available OBJETIVO: Verificar a influência da permanência em temperatura ambiente na análise da transportabilidade por ação ciliar e por tosse e do ângulo de contato do muco traqueobrônquico. MÉTODOS: Foi coletado muco hialino de 30 indivíduos sem doença pulmonar, e purulento de vinte pacientes com bronquiectasia. As amostras foram analisadas logo após a coleta e novamente após 24 h. RESULTADOS: Para o muco purulento, após 24 h em temperatura ambiente, houve aumento no deslocamento por tosse (96 ± 50 vs. 118 ± 61 mm e diminuição do ângulo de contato (32 ± 6 vs. 27 ± 6 graus (p OBJECTIVE: To evaluate the effect that maintaining tracheobronchial sputum at room temperature has on the analysis of ciliary transport and cough, as well as on the contact angle. METHODS: Hyaline sputum was collected from 30 individuals without pulmonary diseases, and purulent sputum was collected from patients with bronchiectasis. The samples were analyzed immediately after collection and again after 24 h. RESULTS: After 24 h at room temperature, the purulent sputum presented an increase in cough-induced dislodgment (96 ± 50 vs. 118 ± 61 mm and a decrease in the contact angle (32 ± 6 vs. 27 ± 6 degrees (p < 0.05. For the hyaline sputum, there were no alterations in the parameters analyzed. CONCLUSION: Hyaline tracheobronchial sputum can be stored in room temperature for 24 h without presenting alterations in ciliary transport or contact angle. However, purulent sputum should not be stored at room temperature for many hours, since ciliary transport and contact angle might be altered as a result.

Color Doppler evaluation of the ocular arterial flow changes in chronic obstructive pulmonary disease

International Nuclear Information System (INIS)

Ozer, Tulay; Altin, Remzi; Ugurbas, Suat Hayri; Ozer, Yetkin; Mahmutyazicioglu, Kamran; Kart, Levent

2006-01-01

Purpose: To evaluate the hemodynamic changes in the extraocular orbital vessels of the patients with chronic obstructive pulmonary disease (COPD), using color Doppler ultrasonography (CDU) technique, and to compare the results with those of healthy control subjects. Methods: Forty-five patients with COPD and 17 healthy control subjects were included in this study. Patients with COPD were classified according to the Global Initiative for Chronic Obstructive Lung Disease (GOLD). Fifteen patients of stage I COPD (mild airflow limitation), stage II COPD (worsening airflow limitation) or stage III COPD (severe airflow limitation) were enrolled into Group I, II and III, respectively. End tidal carbon dioxide (EtCO 2 ), peripheral oxygen saturation (SpO 2 ), pulse rate (PR) and respiratory rate (RR) were measured by using capnograph/pulse oximeter in all patients. Measurements were performed in only one randomly chosen eye of each participant. The peak systolic velocity (PSV), end-diastolic velocity (EDV), and resistance index (RI) were measured in the ophthalmic artery (OA), central retinal artery (CRA), lateral short posterior ciliary artery (LPCA) and medial short posterior ciliary artery (MPCA), using CDU technique. Results: The PSV measurements of the OA were significantly higher in Groups II and III compared to control group (p 2 with PSV (r = 0.53, p < 0.01) and EDV (r = 0.51, p < 0.01) of the OA. Statistically significant correlations were also found for the SpO2 with RI (r = -0.34, p < 0.05) in the OA. Conclusion: We concluded that COPD is associated with impaired retrobulbar hemodynamics, especially in the ophthalmic artery. Moreover, central retinal and posterior ciliary arteries with increased resistance are also found to be affected when compared with healthy control eyes

Essential oil of some seasonal flowering plants grown in Saudi Arabia

OpenAIRE

S.A. Al-Mazroa; L.H. Al-Wahaibi; A.A. Mousa; H.Z. Al-Khathlan

2015-01-01

The constituents of the essential oils of Rumex vesicarius, Erucaria hispanica, Schimpera arabica, Savignya parviflora, Horwoodia dicksoniae, Sisymbrium irio, Plantago amplexicaulis, Plantago boissieri, Arnebia linearifolia, Foeniculum vulgare, Trigonella hamosa, Lotus halophilus, Reseda muricata, Cenchrus ciliaris is reported. These oils were analyzed by GC/MS and most of them are being studied for the first time.

The Practical Aspects of the Visual Act in Studying.

Science.gov (United States)

Robinson, Bernard N.

This paper explores the role of vision in reading and studying. "How Vision Occurs" discusses the functions of rods, cones, the cerebral cortex, and the receptor senses. "The Optometrist and the Educator" views the role of the optometrist in relation to student learning. "Eye Anatomy Terms" defines the ciliary muscle, optic disc and nerve, macula…

Assembly of primary cilia

DEFF Research Database (Denmark)

Pedersen, Lotte B; Veland, Iben R; Schrøder, Jacob M

2008-01-01

knowledge about IFT is based on studies performed in Chlamydomonas and Caenorhabditis elegans. Therefore, our review of the IFT literature includes studies performed in these two model organisms. The role of several non-IFT proteins (e.g., centrosomal proteins) in the ciliary assembly process is also...... discussed. Developmental Dynamics, 2008. (c) 2008 Wiley-Liss, Inc....

Information privacy fundamentals for librarians and information professionals

CERN Document Server

Givens, Cherie L

2014-01-01

This book introduces library and information professionals to information privacy, provides an overview of information privacy in the library and information science context, U.S. privacy laws by sector, information privacy policy, and key considerations when planning and creating a privacy program.

About the value of Ruthenium 106 brachytherapy in the treatment of uveal melanomas

International Nuclear Information System (INIS)

Langmann, G.; Mosboeck, G.; Stuecklschwaiger, G.; Muellner, K.; Lechner, H.; Faulborn, J.

2002-01-01

Background: to investigate the clinical course, sequelae and visual function of uveal melanomas treated with Ruthenium 106 brachytherapy. Patients and method: 47 patients who underwent Ruthenium 106 brachytherapy between 1985 and 2000 were evaluated using Kaplan Meier statistical method. Mean follow up interval was 22 month (range 8 - 152 months). Results: Local tumor control rate was 85 %, 5 years possibility to avoid enucleation was 75 %. The most important sequelae were radiation optic neuropathy (29 %), maculopathy (37 %) and radiation retinopathy (32 %). After terminating the study the 34 % of the patients achieved a visual acuity of 20/40 and more, another 34 % had a visual function of 20/200 and lower. Conclusion: Ruthenium 106 brachytherapy is our method of choice in small medium sized uveal melanomas and a maximum tumor prominence of 6 mm. Tumors have to be located in the midperiphery and outer periphery of the fundus including the ciliary body. In addition to the indications introduced by Lommatzsch we treated ciliary body melanomas with a tumor base more than 3 clock hours (by shifting the plaque) as an alternative therapy to enucleation. (author)

Induced tubulin synthesis is caused by induced gene transcription in Tetrahymena

International Nuclear Information System (INIS)

Seyfert, H.M.; Kohle, D.; Jenovai, S.

1987-01-01

Tubulin synthesis and tubulin mRNA concentrations increase to variable extents during ciliary regeneration in the ciliate Tetrahymena. Experiments described here were carried out to determine whether the increased tubulin mRNa concentrations are due to induced transcription of tubulin genes or to stabilization of tubulin mRNA. In vivo labeling experiments with [ 3 H]uridine and in vitro transcription assays suggest that under conditions of increased protein and tubulin synthesis the rate of transcription is enhanced. Hybridization assays of in vitro transcribed RNA also demonstrate qualitatively that the tubulin genes are transcribed at higher rates when tubulin synthesis is stimulated during ciliary regeneration. This observation is supported by measurements of the half-life of tubulin mRNA molecules in nondeciliated cells: This is approximately 2 h. Since the concentration of tubulin mRNA in cells engaged in cilia regeneration increases from 5 to 19-fold during the first hour of the regeneration period, even a complete stabilization of the tubulin mRNA molecules could not account for an increase in tubulin mRNA concentration of this magnitude

Three types of ependymal cells with intracellular calcium oscillation are characterized by distinct cilia beating properties.

Science.gov (United States)

Liu, Tongyu; Jin, Xingjian; Prasad, Rahul M; Sari, Youssef; Nauli, Surya M

2014-09-01

Ependymal cells are multiciliated epithelial cells that line the ventricles in the adult brain. Abnormal function or structure of ependymal cilia has been associated with various neurological deficits. For the first time, we report three distinct ependymal cell types, I, II, and III, based on their unique ciliary beating frequency and beating angle. These ependymal cells have specific localizations within the third ventricle of the mouse brain. Furthermore, neither ependymal cell types nor their localizations are altered by aging. Our high-speed fluorescence imaging analysis reveals that these ependymal cells have an intracellular pacing calcium oscillation property. Our study further shows that alcohol can significantly repress the amplitude of calcium oscillation and the frequency of ciliary beating, resulting in an overall decrease in volume replacement by the cilia. Furthermore, the pharmacological agent cilostazol could differentially increase cilia beating frequency in type II, but not in type I or type III, ependymal cells. In summary, we provide the first evidence of three distinct types of ependymal cells with calcium oscillation properties. © 2014 Wiley Periodicals, Inc.

Chemical-Free Technique to Study the Ultrastructure of Primary Cilium.

Science.gov (United States)

Mohieldin, Ashraf M; AbouAlaiwi, Wissam A; Gao, Min; Nauli, Surya M

2015-11-02

A primary cilium is a hair-like structure with a width of approximately 200 nm. Over the past few decades, the main challenge in the study of the ultrastructure of cilia has been the high sensitivity of cilia to chemical fixation, which is required for many imaging techniques. In this report, we demonstrate a combined high-pressure freezing (HPF) and freeze-fracture transmission electron microscopy (FFTEM) technique to examine the ultrastructure of a cilium. Our objective is to develop an optimal high-resolution imaging approach that preserves cilia structures in their best natural form without alteration of cilia morphology by chemical fixation interference. Our results showed that a cilium has a swelling-like structure (termed bulb), which was previously considered a fixation artifact. The intramembrane particles observed via HPF/FFTEM indicated the presence of integral membrane proteins and soluble matrix proteins along the ciliary bulb, which is part of an integral structure within the ciliary membrane. We propose that HPF/FFTEM is an important and more suitable chemical-free method to study the ultrastructure of primary cilia.

In Silico and Fluorescence In Situ Hybridization Mapping Reveals Collinearity between the Pennisetum squamulatum Apomixis Carrier-Chromosome and Chromosome 2 of Sorghum and Foxtail Millet.

Directory of Open Access Journals (Sweden)

Sirjan Sapkota

Full Text Available Apomixis, or clonal propagation through seed, is a trait identified within multiple species of the grass family (Poaceae. The genetic locus controlling apomixis in Pennisetum squamulatum (syn Cenchrus squamulatus and Cenchrus ciliaris (syn Pennisetum ciliare, buffelgrass is the apospory-specific genomic region (ASGR. Previously, the ASGR was shown to be highly conserved but inverted in marker order between P. squamulatum and C. ciliaris based on fluorescence in situ hybridization (FISH and varied in both karyotype and position of the ASGR on the ASGR-carrier chromosome among other apomictic Cenchrus/Pennisetum species. Using in silico transcript mapping and verification of physical positions of some of the transcripts via FISH, we discovered that the ASGR-carrier chromosome from P. squamulatum is collinear with chromosome 2 of foxtail millet and sorghum outside of the ASGR. The in silico ordering of the ASGR-carrier chromosome markers, previously unmapped in P. squamulatum, allowed for the identification of a backcross line with structural changes to the P. squamulatum ASGR-carrier chromosome derived from gamma irradiated pollen.

In Silico and Fluorescence In Situ Hybridization Mapping Reveals Collinearity between the Pennisetum squamulatum Apomixis Carrier-Chromosome and Chromosome 2 of Sorghum and Foxtail Millet.

Science.gov (United States)

Sapkota, Sirjan; Conner, Joann A; Hanna, Wayne W; Simon, Bindu; Fengler, Kevin; Deschamps, Stéphane; Cigan, Mark; Ozias-Akins, Peggy

2016-01-01

Apomixis, or clonal propagation through seed, is a trait identified within multiple species of the grass family (Poaceae). The genetic locus controlling apomixis in Pennisetum squamulatum (syn Cenchrus squamulatus) and Cenchrus ciliaris (syn Pennisetum ciliare, buffelgrass) is the apospory-specific genomic region (ASGR). Previously, the ASGR was shown to be highly conserved but inverted in marker order between P. squamulatum and C. ciliaris based on fluorescence in situ hybridization (FISH) and varied in both karyotype and position of the ASGR on the ASGR-carrier chromosome among other apomictic Cenchrus/Pennisetum species. Using in silico transcript mapping and verification of physical positions of some of the transcripts via FISH, we discovered that the ASGR-carrier chromosome from P. squamulatum is collinear with chromosome 2 of foxtail millet and sorghum outside of the ASGR. The in silico ordering of the ASGR-carrier chromosome markers, previously unmapped in P. squamulatum, allowed for the identification of a backcross line with structural changes to the P. squamulatum ASGR-carrier chromosome derived from gamma irradiated pollen.

Neuronal NOS localises to human airway cilia.

Science.gov (United States)

Jackson, Claire L; Lucas, Jane S; Walker, Woolf T; Owen, Holly; Premadeva, Irnthu; Lackie, Peter M

2015-01-30

Airway NO synthase (NOS) isoenzymes are responsible for rapid and localised nitric oxide (NO) production and are expressed in airway epithelium. We sought to determine the localisation of neuronal NOS (nNOS) in airway epithelium due to the paucity of evidence. Sections of healthy human bronchial tissue in glycol methacrylate resin and human nasal polyps in paraffin wax were immunohistochemically labelled and reproducibly demonstrated nNOS immunoreactivity, particularly at the proximal portion of cilia; this immunoreactivity was blocked by a specific nNOS peptide fragment. Healthy human epithelial cells differentiated at an air-liquid interface (ALI) confirmed the presence of all three NOS isoenzymes by immunofluorescence labelling. Only nNOS immunoreactivity was specific to the ciliary axonemeand co-localised with the cilia marker β-tubulin in the proximal part of the ciliary axoneme. We report a novel localisation of nNOS at the proximal portion of cilia in airway epithelium and conclude that its independent and local regulation of NO levels is crucial for normal cilia function. Copyright © 2014 Elsevier Inc. All rights reserved.

Information Retrieval Methods in Libraries and Information Centers ...

African Journals Online (AJOL)

The volumes of information created, generated and stored are immense that without adequate knowledge of information retrieval methods, the retrieval process for an information user would be cumbersome and frustrating. Studies have further revealed that information retrieval methods are essential in information centers ...

Computing handbook information systems and information technology

CERN Document Server

Topi, Heikki

2014-01-01

Disciplinary Foundations and Global ImpactEvolving Discipline of Information Systems Heikki TopiDiscipline of Information Technology Barry M. Lunt and Han ReichgeltInformation Systems as a Practical Discipline Juhani IivariInformation Technology Han Reichgelt, Joseph J. Ekstrom, Art Gowan, and Barry M. LuntSociotechnical Approaches to the Study of Information Systems Steve Sawyer and Mohammad Hossein JarrahiIT and Global Development Erkki SutinenUsing ICT for Development, Societal Transformation, and Beyond Sherif KamelTechnical Foundations of Data and Database ManagementData Models Avi Silber

Information Interaction: Providing a Framework for Information Architecture.

Science.gov (United States)

Toms, Elaine G.

2002-01-01

Discussion of information architecture focuses on a model of information interaction that bridges the gap between human and computer and between information behavior and information retrieval. Illustrates how the process of information interaction is affected by the user, the system, and the content. (Contains 93 references.) (LRW)

Information Impact | Journal of Information and Knowledge ...

African Journals Online (AJOL)

Information Impact | Journal of Information and Knowledge Management

Information Impact | Journal of Information and Knowledge Management. 77 ... Libraries all over the world are applying information and communication ..... Bank Branch Performance, The International Journal on Advances in ICT for Emerging.

Information Crisis

CERN Document Server

Losavio, Michael

2012-01-01

Information Crisis discusses the scope and types of information available online and teaches readers how to critically assess it and analyze potentially dangerous information, especially when teachers, editors, or other information gatekeepers are not available to assess the information for them. Chapters and topics include:. The Internet as an information tool. Critical analysis. Legal issues, traps, and tricks. Protecting personal safety and identity. Types of online information.

Induced Pluripotent Stem Cells: Generation, Characterization, and Differentiation--Methods and Protocols.

Science.gov (United States)

Graversen, Veronica Kon; Chavala, Sai H

2016-01-01

Reprogramming fibroblasts into induced pluripotent stem cells (iPSC) remains a promising technique for cell replacement therapy. Diverse populations of somatic cells have been examined for their reprogramming potential. Recently, ocular ciliary body epithelial cells (CECs) have been reprogrammed with high reprogramming efficiency and single transcription factor reprogramming, making them an exciting candidate for cellular reprogramming strategies.

Essential oil of some seasonal flowering plants grown in Saudi Arabia

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S.A. Al-Mazroa

2015-03-01

Full Text Available The constituents of the essential oils of Rumex vesicarius, Erucaria hispanica, Schimpera arabica, Savignya parviflora, Horwoodia dicksoniae, Sisymbrium irio, Plantago amplexicaulis, Plantago boissieri, Arnebia linearifolia, Foeniculum vulgare, Trigonella hamosa, Lotus halophilus, Reseda muricata, Cenchrus ciliaris is reported. These oils were analyzed by GC/MS and most of them are being studied for the first time.

The effects of microwave radiation on rabbit's retina

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Mohammad R. Talebnejad

2018-03-01

Conclusions: Histopathologically, cell phone simulated MW irradiation had no significant detrimental effect on the retina. However, ciliary body congestion was observed in greater fraction of those who received higher MW doses. Although there was no significant difference between post-treatment mean ERG values, there were statistically non-significant trends toward greater changes in the MW irradiated eyes.

The Information Tekhnology Share In Management Information System

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Nur Zeina Maya Sari

2015-08-01

Full Text Available Abstract Management Information System growth cause change of role from all manager in decision making the information technology. While prima facie reason for the usage of information technology in business to support such a manner so that information system may operate better OBrienamp Marakas 2004. Its meaning with existence of information tekhnology in management information system SIM company management decision making which initially often pursued by many factor of non technical become accurately is relevant complete and on schedule

Developing geographic information infrastructure : The role of information policies

NARCIS (Netherlands)

Van Loenen, B.

2006-01-01

Within information societies, information availability is a key issue affecting societyâs well being. The infrastructure underlying the foundation of the information society may be referred to as the information infrastructure. A geographic information infrastructure (GII) supports the information

Inform: Efficient Information-Theoretic Analysis of Collective Behaviors

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Douglas G. Moore

2018-06-01

Full Text Available The study of collective behavior has traditionally relied on a variety of different methodological tools ranging from more theoretical methods such as population or game-theoretic models to empirical ones like Monte Carlo or multi-agent simulations. An approach that is increasingly being explored is the use of information theory as a methodological framework to study the flow of information and the statistical properties of collectives of interacting agents. While a few general purpose toolkits exist, most of the existing software for information theoretic analysis of collective systems is limited in scope. We introduce Inform, an open-source framework for efficient information theoretic analysis that exploits the computational power of a C library while simplifying its use through a variety of wrappers for common higher-level scripting languages. We focus on two such wrappers here: PyInform (Python and rinform (R. Inform and its wrappers are cross-platform and general-purpose. They include classical information-theoretic measures, measures of information dynamics and information-based methods to study the statistical behavior of collective systems, and expose a lower-level API that allow users to construct measures of their own. We describe the architecture of the Inform framework, study its computational efficiency and use it to analyze three different case studies of collective behavior: biochemical information storage in regenerating planaria, nest-site selection in the ant Temnothorax rugatulus, and collective decision making in multi-agent simulations.

Information barriers for the protection of sensitive information

International Nuclear Information System (INIS)

Dougan, A.D.; Dunn, J.; Seager, K.; Smith, M.; Beach, D.; Clinton, J.; Vanier, P.

2013-01-01

An Information Barrier is a combination of technology and procedures that prevent the release of a host country's sensitive information to a monitoring party during an inspection of a sensitive item, while enabling assurance of an accurate assessment of host country declarations regarding the item. Information barriers, as a concept, arose from the need to balance two competing requirements - specifically, certification of information protection by a host country and authentication of measurement and information accuracy by a monitoring party. The U.S. Department of Energy's National Nuclear Security Administration, in conjunction with several U.S. National Laboratories, has explored information barrier development in detail, and has continued the evolution of essential work done by the U.S. Information Barrier Working Group beginning in the late 1990's, and other international efforts, including the U.S.-Russia-IAEA Trilateral Initiative. This paper explains the rationale for information barriers, and explores the development and application of information barrier concepts for potential future arms control initiatives, including recent work and advances in capabilities. It also considers applications of information barrier concepts applied in other fields.The paper is followed by the slides of the presentation. (authors)

Information management

Science.gov (United States)

Ricks, Wendell; Corker, Kevin

1990-01-01

Primary Flight Display (PFD) information management and cockpit display of information management research is presented in viewgraph form. The information management problem in the cockpit, information management burdens, the key characteristics of an information manager, the interface management system handling the flow of information and the dialogs between the system and the pilot, and overall system architecture are covered.

Correlation Between Information Technology And Management Information Systems Quality

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Annisa Fitri Anggraeni

2015-08-01

Full Text Available The aim of this research is to find out the correlation between Information Technology and Management Information System Quality. This research applies literature review from various theories and previously conducted research where controversies between Information Technology and Management Information Systems. The result of this research shows that Information Technology is a part of Management Information System and supports the performance of Management Information Systems.

Agricultural informational flow in informal communication networks ...

African Journals Online (AJOL)

Agricultural informational flow in informal communication networks of farmers in Ghana. ... should identify such farmers who can serve as intermediaries between actors to help disseminate information in rural communities. Keywords: key communicators, farmers, rural communities, social networks, extension agents ...

How Informed Are Informal Educators?

Science.gov (United States)

Lederman, Norman G.; Niess, Margaret L.

1998-01-01

Explores current reforms in both mathematics and science education that emphasize the importance of learning in informal settings. Suggests that informal education must include planned and purposeful attempts to facilitate students' understanding of mathematics and science in community settings other than the local school. (Author/CCM)

Ciliary heterogeneity within a single cell: the Paramecium model.

Science.gov (United States)

Aubusson-Fleury, Anne; Cohen, Jean; Lemullois, Michel

2015-01-01

Paramecium is a single cell able to divide in its morphologically differentiated stage that has many cilia anchored at its cell surface. Many thousands of cilia are thus assembled in a short period of time during division to duplicate the cell pattern while the cell continues swimming. Most, but not all, of these sensory cilia are motile and involved in two main functions: prey capture and cell locomotion. These cilia display heterogeneity, both in their length and their biochemical properties. Thanks to these properties, as well as to the availability of many postgenomic tools and the possibility to follow the regrowth of cilia after deciliation, Paramecium offers a nice opportunity to study the assembly of the cilia, as well as the genesis of their diversity within a single cell. In this paper, after a brief survey of Paramecium morphology and cilia properties, we describe the tools and the protocols currently used for immunofluorescence, transmission electron microscopy, and ultrastructural immunocytochemistry to analyze cilia, with special recommendations to overcome the problem raised by cilium diversity. Copyright © 2015. Published by Elsevier Inc.

Regulation of the branchial ciliary activity in the mussel

NARCIS (Netherlands)

Dral, A.D.G.

1977-01-01

In mussels the movement of the cilia on the gill are basically autonomous and influenced by environmental factors. The branchial nerve has an inhibitory as well as a stimulating effect on the activity of the lateral cilia. The reactions of these cilia to changing temperature and chlorinity in

The information activity of rail passenger information staff: a foundation for information system requirements

Directory of Open Access Journals (Sweden)

Martin Rose

2006-01-01

Full Text Available Introduction. This paper examines the goal-directed information activity of passenger information staff, working in the dynamic environment of rail network control. The explicit aim is to define a meaningful set of information system requirements. The report shows how dynamic situations may lead us to question a number of established theories of information science. Method. Passenger information officers (PIOs were observed on duty within the rail command and control headquarters. Observation sessions totally eight hours involved the manual recording of sequential information flows and the associated activity of PIOs. A semi-structured management interview was also conducted to provide further insight into the organizational context. Analysis. A viewpoint-oriented analysis technique was used to analyse sequential data captured during observation sessions. Event sequences that represent and explain the viewpoints were identified and elaborated into detailed scenario descriptions. Results. The analysis both supports and contests a number of established theories from information science. Additionally, a range of 'mandatory' and 'desirable' system requirements are derived from the scenario and viewpoint analyses. Conclusion. Dynamic situations have a significant impact on information behaviour which is not always predicted by current theories of information science.

Information Impact: Journal of Information and Knowledge ...

African Journals Online (AJOL)

Information Impact: Journal of Information and Knowledge Management

Information Impact: Journal of Information and Knowledge Management. 55. Adeola Esther Olutoki ... The main instruments used for data collection were research ... collections in the library and enhance academic performance. ... libraries; they contain the latest information on research findings which are of great importance.

Biological information systems: Evolution as cognition-based information management.

Science.gov (United States)

Miller, William B

2018-05-01

An alternative biological synthesis is presented that conceptualizes evolutionary biology as an epiphenomenon of integrated self-referential information management. Since all biological information has inherent ambiguity, the systematic assessment of information is required by living organisms to maintain self-identity and homeostatic equipoise in confrontation with environmental challenges. Through their self-referential attachment to information space, cells are the cornerstone of biological action. That individualized assessment of information space permits self-referential, self-organizing niche construction. That deployment of information and its subsequent selection enacted the dominant stable unicellular informational architectures whose biological expressions are the prokaryotic, archaeal, and eukaryotic unicellular forms. Multicellularity represents the collective appraisal of equivocal environmental information through a shared information space. This concerted action can be viewed as systematized information management to improve information quality for the maintenance of preferred homeostatic boundaries among the varied participants. When reiterated in successive scales, this same collaborative exchange of information yields macroscopic organisms as obligatory multicellular holobionts. Cognition-Based Evolution (CBE) upholds that assessment of information precedes biological action, and the deployment of information through integrative self-referential niche construction and natural cellular engineering antecedes selection. Therefore, evolutionary biology can be framed as a complex reciprocating interactome that consists of the assessment, communication, deployment and management of information by self-referential organisms at multiple scales in continuous confrontation with environmental stresses. Copyright © 2017 Elsevier Ltd. All rights reserved.

College Students’ Information Needs and Information Seeking Behaviors regarding Personal Information

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Yu-Wen Liu

2017-12-01

Full Text Available This study analyzed college students’ reactions toward the issues of personal information. Students’ needs and seeking behaviors for personal information were assessed. Relevant literature was reviewed for framing the research questions and designing the questionnaire items for survey. Survey subjects were students from an university at northern Taiwan. A set of questionnaire items were used to collect research data. Statistical analysis from 252 valid data reveals some items were highly rated: Students reflected highly for their need of knowledge under the security threat of personal information (M = 4.29. They reacted strongly on acquiring knowledge and resources through the Internet (M = 4.24. They preferred the use of resources clear and easy to be understood (M = 4.04. However, most students had low level faith toward either government or non-governmental organizations in securing their personal information (M < 3.0 for most items. More effort among education and government should be emphasized in the future to improve personal use and reduce uncertainty in the use of personal information.

INFORMATION CULTURE AND INFORMATION SAFETY OF SCHOOLCHILDREN

Directory of Open Access Journals (Sweden)

E. G. Belyakova

2017-01-01

Full Text Available Introduction. The article is devoted to the problem of interaction between schoolchildren and possible informational risks transmitted on the Internet. Considering the lack of external filters on the way of harmful information streams, it is actually necessary to develop information culture of schoolchildren, their abilities to sensibly and critically interpret the information on the Internet, and choice of adequate behaviour models surfing the Web. The aim of the present research is to analyze the state of informational safety of schoolchildren while using the Internet; gaining an understanding of the role of external restrictions and opportunities of intrapersonal filtration of the harmful Internet content depending on children age. Methodology and research methods. The methodology of the research is based on modern methods aimed to consider the problem of personal socialization in modern information society. Thus, the Internet Initiatives Development Fund (IIDF questionnaire let the authors define the level of awareness of recipients on the problem under consideration. Results and scientific novelty. The theoretical analysis helped the authors predict the correlation of basic methods in order to guarantee personal safety of schoolchildren taking into account the process of maturity as well as the decrease of external filters that may stop harmful content. Empirical part of the research has enabled to reveal decrease in external control of staying of a child in network in the process of growing up against the background of restrictive attitudes prevalence among teachers and parents. Therefore, the research supposed to improve information culture of schoolchildren from the earliest ages encouraging them to sensibly and correctly interpret the information on the Internet. Practical significance. The practical recommendations to parents and teachers in order to improve informational personal safety of schoolchildren are proposed. The relevancy

Rethinking information handling: designing for information offload

DEFF Research Database (Denmark)

Hansen, Lone Koefoed; Pedersen, Peter Dalsgård; Eriksson, Eva

2005-01-01

This paper introduces information offload as a supplement way of responding to and designing for information. By presenting iFlush, a design concept aiming at providing the user with a possibility to offload information and then instantly dispose of it, the paper presents an approach towards desi...... designing for reflection and also serves as a critique towards information overload. Furthermore, the absence of reflection on the role played by HCI community in the constant augmenting of all spaces is addressed....

Social information

Directory of Open Access Journals (Sweden)

Luiz Fernando de Barros Campos

Full Text Available Based on Erving Goffman's work, the article aims to discuss a definition of information centered on the type conveyed by individuals in a multimodal way, encompassing language and body in situations of co-presence, where face-to-face interaction occurs, and influencing inter-subjective formation of the self. Six types of information are highlighted: material information, expressive information, ritualized information, meta-information, strategic information, and information displays. It is argued that the construction of this empirical object tends to dissolve the tension among material, cognitive and pragmatic aspects, constituting an example of the necessary integration among them. Some vulnerable characteristics of the theory are critically mentioned and it is suggested that the concept of information displays could provide a platform to approach the question of the interaction order in its relations with the institutional and social orders, and consequently, to reassess the scope of the notion of social information analyzed.

Information Needs, Sources, and Information Seeking Behaviour of ...

African Journals Online (AJOL)

This work which focused on the policy-makers/administrators as information users was carried out using hundred (100) questionnaire in order to ascertain the information needs, sources and information seeking behaviour of the administrators. The barrier to information flow to them was also identified. The study adopted a ...

Information Assurance Security in the Information Environment

CERN Document Server

Blyth, Andrew

2006-01-01

Intended for IT managers and assets protection professionals, this work aims to bridge the gap between information security, information systems security and information warfare. It covers topics such as the role of the corporate security officer; Corporate cybercrime; Electronic commerce and the global marketplace; Cryptography; and, more.

Communicating stereotype-relevant information: is factual information subject to the same communication biases as fictional information?

Science.gov (United States)

Goodman, Ruth L; Webb, Thomas L; Stewart, Andrew J

2009-07-01

Factual information is more frequently read and discussed than fictional information. However, research on the role of communication in shaping stereotypes has focused almost exclusively on fictional narratives. In Experiments 1 and 2 a newspaper article containing information about heroin users was communicated along chains of 4 people. No stereotype-consistency bias was observed. Instead, a greater proportion of stereotype-inconsistent information was communicated than was stereotype-consistent or -neutral information. Three further experiments investigated explanations for the difference between the communication of fictional and factual information. Experiment 3 ruled out the possibility that participants' beliefs about the validity of the information could influence the way that it is communicated. Experiments 4 and 5 divided information into concrete (a specific event or fact) or abstract (opinion). A stereotype-consistency bias emerged only for abstract information. In summary, linguistic abstraction moderates whether stereotype-consistency biases emerge in the communication of stereotype-relevant factual information.

Information technology acceptance in health information management.

Science.gov (United States)

Abdekhoda, M; Ahmadi, M; Dehnad, A; Hosseini, A F

2014-01-01

User acceptance of information technology has been a significant area of research for more than two decades in the field of information technology. This study assessed the acceptance of information technology in the context of Health Information Management (HIM) by utilizing Technology Acceptance Model (TAM) which was modified and applied to assess user acceptance of health information technology as well as viability of TAM as a research construct in the context of HIM. This was a descriptive- analytical study in which a sample of 187 personnel from a population of 363 personnel, working in medical records departments of hospitals affiliated to Tehran University of Medical Sciences, was selected. Users' perception of applying information technology was studied by a researcher-developed questionnaire. Collected data were analyzed by SPSS software (version16) using descriptive statistics and regression analysis. The results suggest that TAM is a useful construct to assess user acceptance of information technology in the context of HIM. The findings also evidenced the perceived ease of use (PEOU) and perceived usefulness (PE) were positively associated with favorable users' attitudes towards HIM. PU was relatively more associated (r= 0.22, p = 0.05) than PEOU (r = 0.014, p = 0.05) with favorable user attitudes towards HIM. Users' perception of usefulness and ease of use are important determinants providing the incentive for users to accept information technologies when the application of a successful HIM system is attempted. The findings of the present study suggest that user acceptance is a key element and should subsequently be the major concern of health organizations and health policy makers.

108 Information Retrieval Methods in Libraries and Information ...

African Journals Online (AJOL)

User

without adequate knowledge of information retrieval methods, the retrieval process for an ... discusses the concept of Information retrieval, the various information ..... Other advantages of automatic indexing are the maintenance of consistency.

Informational Urbanism

Directory of Open Access Journals (Sweden)

Wolfgang G. Stock

2015-10-01

Full Text Available Contemporary and future cities are often labeled as "smart cities," "ubiquitous cities," "knowledge cities" and "creative cities." Informational urbanism includes all aspects of information and knowledge with regard to urban regions. "Informational city" is an umbrella term uniting the divergent trends of information-related city research. Informational urbanism is an interdisciplinary endeavor incorporating on the one side computer science and information science and on the other side urbanism, architecture, (city economics, and (city sociology. In our research project on informational cities, we visited more than 40 metropolises and smaller towns all over the world. In this paper, we sketch the theoretical background on a journey from Max Weber to the Internet of Things, introduce our research methods, and describe main results on characteristics of informational cities as prototypical cities of the emerging knowledge society.

Information Concepts

CERN Document Server

Marchionini, Gary

2010-01-01

Information is essential to all human activity, and information in electronic form both amplifies and augments human information interactions. This lecture surveys some of the different classical meanings of information, focuses on the ways that electronic technologies are affecting how we think about these senses of information, and introduces an emerging sense of information that has implications for how we work, play, and interact with others. The evolutions of computers and electronic networks and people's uses and adaptations of these tools manifesting a dynamic space called cyberspace. O

A System Approach to Navy Medical Education and Training. Appendix 32- Competency Curricula for Ophthalmic Clinic Assistant and Ophthalmic Technician

Science.gov (United States)

1974-08-31

chamber fluid, and gonioscopy exam (Conditions) With direct supervision (Criteria) In accordance with physician’s instructions (Consequence) Completion of...posterior chambers Gonioscopy techniques Slit-lamp biomicroscopy techniques Smear and culture techniques Accuracy in recording ard charting Manual...CILIARY BODY/ANGLE STRUCTURE TESTS I TASXS a. Assist with gonioscopy PERFO-RANCE OBJECTIVE (Stimulus) Upon physician’s orders (Behavior) The OPHTA

Usher syndrome type I associated with bronchiectasis and immotile nasal cilia in two brothers.

OpenAIRE

Bonneau, D; Raymond, F; Kremer, C; Klossek, J M; Kaplan, J; Patte, F

1993-01-01

Usher syndrome type I is an autosomal recessive disease characterised by congenital sensorineural deafness, involvement of the vestibular system, and progressive visual loss owing to retinitis pigmentosa. Here we report the association of this disease with bronchiectasis, chronic sinusitis, and reduced nasal mucociliary clearance in two sibs and we suggest Usher syndrome type I could be a primary ciliary disorder.

Floridi's Philosophy of Information and Information Ethics

DEFF Research Database (Denmark)

Ess, Charles

2009-01-01

In order to evaluate Floridi's philosophy of information (PI) and correlative information ethics (IE) as potential frameworks for a global information and computing ethics (ICE), I review a range of important criticisms, defenses, and extensions of PI and IE, along with Floridi's responses to these......, as gathered together in a recent special issue of Ethics and Information Technology. A revised and expanded version of PI and IE emerges here, one that brings to the foreground PI's status as a philosophical naturalism—one with both current application and important potential in the specific domains...... of privacy and information law. Further, the pluralism already articulated by Floridi in his PI is now more explicitly coupled with an ethical pluralism in IE that will be enhanced through IE's further incorporation of discourse ethics. In this form, PI and IE emerge as still more robust frameworks...

Language as information system: transfer and saving of the information

Directory of Open Access Journals (Sweden)

Irina Mikhaylovna Nekipelova

2015-11-01

Full Text Available The paper is devoted to research of the language system as an information system. The language is not the information itself. It contains information minimum and has information restrictions. Under the information should be understood texts and speech, created by using a language. Modern forms of existence of living natural language are distributed databases with multiple accesses, because bearers of language constantly and concurrently use it. Language and speech always exchange information, creating a unified information environment, where a human is. At any stage of information exchange the change of self-descriptiveness takes place one way or the other. When a human try to reflect world around him, some part of reflected information is lost due to conversion into language and saving into it in formalized form. These processes of storing information are related to the fact that in the production and perception of the statements optimization mechanisms of human thinking are made. When we perceive information its preservation can happen without information loss and with the loss of information.

Information Myopia

Directory of Open Access Journals (Sweden)

Nadi Helena Presser

2016-04-01

Full Text Available This article reflects on the ways of appropriation in organizations. The notion of Information Myopia is characterized by the lack of knowledge about the available informational capabilities in organizations, revealing a narrow view of the information environment. This analysis has focused on the process for renewing the software licenses contracts of a large multinational group, in order to manage its organizational assets in information technology. The collected, explained and justified information allowed to elaborate an action proposal, which enabled the creation of new organizational knowledge. In its theoretical dimension, the value of information was materialized by its use, in a collective process of organizational learning.

Information cultures

DEFF Research Database (Denmark)

Skouvig, Laura

2017-01-01

The purpose of this article is to suggest a genealogy of the concept of information beyond the 20th century. The article discusses how the concept of information culture might provide a way of formulating such a genealogic strategy. The article approaches this purpose by providing a general...... narrative of premodern information cultures, examining works on early-modern scholars and 18th century savants and discussion of what seems to be a Foucauldian rupture in the conceptualization of information in 19th century England. The findings of the article are situated in the thinking that a genealogy...... of information would reveal that information had specific purposes in specific settings....

Peran Information Conciousness dan Nutrient Information dalam Meningkatkan Kinerja Individual

Directory of Open Access Journals (Sweden)

Niken Wahyu Wilujeng

2014-12-01

Full Text Available The objective of this research is to analyze nutrient information and information consciousness as factors that influence employee’s performance and job satisfication as intervening variable for finance employee in Brawijaya University. By employing Partial Least Square technique, it was indicated that nutrient information and information consciousness have positive effect to job satisfication, while job satisfication can also fully mediate nutrient information and information consciousness to employee’s performance. This research also indicated that information consciousness and nutrient information are factors that influence finance employee’s performance through intelectual emphasis.

Information needs and information seeking behaviour of small-scale ...

African Journals Online (AJOL)

It is thus important for the government to improve access to extension services, and equip them with necessary skills and adequate information resources. ... information needs, map communities' knowledge and information sources, create awareness of information sources, and knowledge culture, and use multiple sources ...

When Information Improves Information Security

Science.gov (United States)

Grossklags, Jens; Johnson, Benjamin; Christin, Nicolas

This paper presents a formal, quantitative evaluation of the impact of bounded-rational security decision-making subject to limited information and externalities. We investigate a mixed economy of an individual rational expert and several naïve near-sighted agents. We further model three canonical types of negative externalities (weakest-link, best shot and total effort), and study the impact of two information regimes on the threat level agents are facing.

The effects of informal carers' characteristics on their information needs: The information needs state approach.

Science.gov (United States)

Alzougool, Basil; Chang, Shanton; Gray, Kathleen

2017-09-01

There has been little research that provides a comprehensive account of the nature and aspects of information needs of informal carers. The authors have previously developed and validated a framework that accounts for major underlying states of information need. This paper aims to apply this framework to explore whether there are common demographic and socioeconomic characteristics that affect the information needs states of carers. A questionnaire about the information needs states was completed by 198 carers above 18 years old. We use statistical methods to look for similarities and differences in respondents' information needs states, in terms of the demographic and socioeconomic variables. At least one information needs state varies among carers, in terms of seven demographic and socioeconomic variables: the age of the patient(s) that they are caring for; the condition(s) of the patient(s) that they are caring for; the number of patients that they are caring for; their length of time as a carer; their gender; the country that they live in; and the population of the area that they live in. The findings demonstrate the utility of the information needs state framework. We outline some practical implications of the framework.

75 FR 28777 - Information Collection; Financial Information Security Request Form

Science.gov (United States)

2010-05-24

... Collection; Financial Information Security Request Form AGENCY: Forest Service, USDA. ACTION: Notice; Request... currently approved information collection; Financial Information Security Request Form. DATES: Comments must... Standard Time, Monday through Friday. SUPPLEMENTARY INFORMATION: Title: Financial Information Security...

An Evaluation of Information Consistency in Grid Information Systems

CERN Document Server

Field, Laurence

2017-01-01

A Grid information system resolves queries that may need to consider all information sources (Grid services), which are widely distributed geographically, in order to enable efficient Grid functions that may utilise multiple cooperating services. Fundamentally this can be achieved by either moving the query to the data (query shipping) or moving the data to the query (data shipping). Existing Grid information system implementations have adopted one of the two approaches. This paper explores the two approaches in further detail by evaluating them to the best possible extent with respect to Grid information system benchmarking metrics. A Grid information system that follows the data shipping approach based on the replication of information that aims to improve the currency for highly-mutable information is presented. An implementation of this, based on an Enterprise Messaging System, is evaluated using the benchmarking method and the consequence of the results for the design of Grid information systems is discu...

Information behaviour of migrant Hispanic farm workers and their families in the Pacific Northwest Information grounds, Information behaviour, Behavior, Washington, Immigrants, Information habits

Directory of Open Access Journals (Sweden)

Karen E. Fisher

2004-01-01

Full Text Available Immigrants are generally perceived to be information poor, meaning they face major challenges with finding and using greatly needed everyday information. However, little research exists from an information behaviour perspective as differences in language, culture, and other factors such as access make immigrants a difficult population to study. We explored the everyday information behaviour and information grounds of migrant Hispanic farm workers through field observation and interviews with users, non-users, and staff of community technology centres in a major agricultural area. Findings suggest that personal networks having various levels of credibility were used more readily than any other type of information source. Credibility and use of various sources seemed to relate to personal status as well as interest in information.

Collecting Information for Rating Global Assessment of Functioning (GAF): Sources of Information and Methods for Information Collection.

Science.gov (United States)

I H, Monrad Aas

2014-11-01

Global Assessment of Functioning (GAF) is an assessment instrument that is known worldwide. It is widely used for rating the severity of illness. Results from evaluations in psychiatry should characterize the patients. Rating of GAF is based on collected information. The aim of the study is to identify the factors involved in collecting information that is relevant for rating GAF, and gaps in knowledge where it is likely that further development would play a role for improved scoring. A literature search was conducted with a combination of thorough hand search and search in the bibliographic databases PubMed, PsycINFO, Google Scholar, and Campbell Collaboration Library of Systematic Reviews. Collection of information for rating GAF depends on two fundamental factors: the sources of information and the methods for information collection. Sources of information are patients, informants, health personnel, medical records, letters of referral and police records about violence and substance abuse. Methods for information collection include the many different types of interview - unstructured, semi-structured, structured, interviews for Axis I and II disorders, semistructured interviews for rating GAF, and interviews of informants - as well as instruments for rating symptoms and functioning, and observation. The different sources of information, and methods for collection, frequently result in inconsistencies in the information collected. The variation in collected information, and lack of a generally accepted algorithm for combining collected information, is likely to be important for rated GAF values, but there is a fundamental lack of knowledge about the degree of importance. Research to improve GAF has not reached a high level. Rated GAF values are likely to be influenced by both the sources of information used and the methods employed for information collection, but the lack of research-based information about these influences is fundamental. Further development of

Information Sharing and Information Acqusition in Credit Markets

NARCIS (Netherlands)

Karapetyan, A.; Stacescu, B.

2009-01-01

Since information asymmetries have been identified as an important source of bank profits, it may seem that the establishment of information sharing arrangements such as credit registers and bureaus will lead to lower investment in acquiring information. However, banks base their decisions on both

Information Architecture in Library and Information Science Curricula.

Science.gov (United States)

Robins, David

2002-01-01

Discusses how information architecture is being handled in some library and information science (LIS) programs and suggests mappings between traditional LIS curricula and the marketplace for information architects. Topics include terminology used in LIS curricula; current job opportunities; and projections for the future. (LRW)

Information Science and Information Systems: Conjunct Subjects Disjunct Disciplines.

Science.gov (United States)

Ellis, David; Allen, David; Wilson, Tom

1999-01-01

Examines the relationship between information science and information-systems (IS) research through analysis of the subject literature of each field and by citation and co-citation analysis of highly cited researchers in each field. Subfields of user studies and information-retrieval research were selected to represent information-science…

Molecular genetics of dyslexia: an overview.

Science.gov (United States)

Carrion-Castillo, Amaia; Franke, Barbara; Fisher, Simon E

2013-11-01

Dyslexia is a highly heritable learning disorder with a complex underlying genetic architecture. Over the past decade, researchers have pinpointed a number of candidate genes that may contribute to dyslexia susceptibility. Here, we provide an overview of the state of the art, describing how studies have moved from mapping potential risk loci, through identification of associated gene variants, to characterization of gene function in cellular and animal model systems. Work thus far has highlighted some intriguing mechanistic pathways, such as neuronal migration, axon guidance, and ciliary biology, but it is clear that we still have much to learn about the molecular networks that are involved. We end the review by highlighting the past, present, and future contributions of the Dutch Dyslexia Programme to studies of genetic factors. In particular, we emphasize the importance of relating genetic information to intermediate neurobiological measures, as well as the value of incorporating longitudinal and developmental data into molecular designs. Copyright © 2013 John Wiley & Sons, Ltd.

Cilia induced cerebrospinal fluid flow in the third ventricle of brain

Science.gov (United States)

Wang, Yong; Westendorf, Christian; Faubel, Regina; Eichele, Gregor; Bodenschatz, Eberhard

2016-11-01

Cerebrospinal fluid (CSF) conveys many physiologically important signaling factors through the ventricles of the mammalian brain. The walls of the ventricles are covered with motile cilia that were thought to generate a laminar flow purely following the curvature of walls. However, we recently discovered that cilia of the ventral third ventricle (v3V) generate a complex flow network along the wall, leading to subdivision of the v3V. The contribution of such cilia induced flow to the overall three dimensional volume flow remains to be investigated by using numerical simulation, arguably the best approach for such investigations. The lattice Boltzmann method is used to study the CFS flow in a reconstructed geometry of the v3V. Simulation of CSF flow neglecting cilia in this geometry confirmed that the previous idea about pure confined flow does not reflect the reality observed in experiment. The experimentally recorded ciliary flow network along the wall was refined with the smoothed particle hydrodynamics and then adapted as boundary condition in simulation. We study the contribution of the ciliary network to overall CSF flow and identify site-specific delivery of CSF constituents with respect to the temporal changes.

WD60/FAP163 is a dynein intermediate chain required for retrograde intraflagellar transport in cilia

Science.gov (United States)

Patel-King, Ramila S.; Gilberti, Renée M.; Hom, Erik F. Y.; King, Stephen M.

2013-01-01

Retrograde intraflagellar transport (IFT) is required for assembly of cilia. We identify a Chlamydomonas flagellar protein (flagellar-associated protein 163 [FAP163]) as being closely related to the D1bIC(FAP133) intermediate chain (IC) of the dynein that powers this movement. Biochemical analysis revealed that FAP163 is present in the flagellar matrix and is actively trafficked by IFT. Furthermore, FAP163 copurified with D1bIC(FAP133) and the LC8 dynein light chain, indicating that it is an integral component of the retrograde IFT dynein. To assess the functional role of FAP163, we generated an RNA interference knockdown of the orthologous protein (WD60) in planaria. The Smed-wd60(RNAi) animals had a severe ciliary assembly defect that dramatically compromised whole-organism motility. Most cilia were present as short stubs that had accumulated large quantities of IFT particle–like material between the doublet microtubules and the membrane. The few remaining approximately full-length cilia had a chaotic beat with a frequency reduced from 24 to ∼10 Hz. Thus WD60/FAP163 is a dynein IC that is absolutely required for retrograde IFT and ciliary assembly. PMID:23864713

Post-secretory fate of host defence components in mucus.

Science.gov (United States)

Salathe, Matthias; Forteza, Rosanna; Conner, Gregory E

2002-01-01

Airway mucus is a complex mixture of secretory products that provide a multifaceted defence against infection. Among many antimicrobial substances, mucus contains a peroxidase identical to milk lactoperoxidase (LPO) that is produced by goblet cells and submucosal glands. Airway secretions contain the substrates for LPO, namely thiocyanate and hydrogen peroxide, at concentrations sufficient for production of the biocidal compound hypothiocyanite, a fact confirmed by us in vitro. In vivo, inhibition of airway LPO in sheep significantly inhibits bacterial clearance, suggesting that the LPO system is a major contributor to host defences. Since secretory products including LPO are believed to be steadily removed by mucociliary clearance, their amount and availability on the surface is thought to be controlled solely by secretion. In contrast to this paradigm, new data suggest that LPO and other substances are retained at the ciliary border of the airway epithelium by binding to surface-associated hyaluronan, thereby providing an apical, fully active enzyme pool. Thus, hyaluronan, secreted from submucosal gland cells, plays a previously unrecognized pivotal role in mucosal host defence by retaining LPO and possibly other substances important for first line host defence at the apical surface 'ready for use' and protected from ciliary clearance.

Photosensory transduction in unicellular eukaryotes: a comparison between related ciliates Blepharisma japonicum and Stentor coeruleus and photoreceptor cells of higher organisms.

Science.gov (United States)

Sobierajska, Katarzyna; Fabczak, Hanna; Fabczak, Stanisław

2006-06-01

Blepharisma japonicum and Stentor coeruleus are related ciliates, conspicuous by their photosensitivity. They are capable of avoiding illuminated areas in the surrounding medium, gathering exclusively in most shaded places (photodispersal). Such behaviour results mainly from motile photophobic response occurring in ciliates. This light-avoiding response is observed during a relatively rapid increase in illumination intensity (light stimulus) and consists of cessation of cell movement, a period of backward movement (ciliary reversal), followed by a forward swimming, usually in a new direction. The photosensitivity of ciliates is ascribed to their photoreceptor system, composed of pigment granules, containing the endogenous photoreceptor -- blepharismin in Blepharisma japonicum, and stentorin in Stentor coeruleus. A light stimulus, applied to both ciliates activates specific stimulus transduction processes leading to the electrical changes at the plasma membrane, correlated with a ciliary reversal during photophobic response. These data indicate that both ciliates Blepharisma japonicum and Stentor coeruleus, the lower eukaryotes, are capable of transducing the perceived light stimuli in a manner taking place in some photoreceptor cells of higher eukaryotes. Similarities and differences concerning particular stages of light transduction in eukaryotes at different evolutional levels are discussed in this article.

Ocular distribution of topically applied adrenaline in albino and pigmented rabbits

International Nuclear Information System (INIS)

Urtti, A.; Periviita, L.; Salminen, L.

1984-01-01

Systemic absorption, ocular distribution and ocular metabolism in albino and pigmented rabbit of topically applied 1% [ 3 H]1-adrenaline eye drops was studied by liquid scintillation counting and thin layer chromatography. The effect of adrenaline on the pupillary diameter was also registered. The peak plasma level of adrenaline was reached at 150 min. The corneal adrenaline metabolism was substantial, but did not vary with the rabbit strain. At 30 min after instillation, the total adrenaline concentration of pigmented iris and ciliary body exceeded that of corresponding albino tissues. At 180 min the drug concentration of the iris and ciliary body did not differ between the rabbit strains. Th time course of adrenaline induced mydriasis was equal in both rabbit strains. Since neither total adrenaline concentration at 180 min nor the time course of adrenaline induced mydriasis differed between the rabbit strains, the higher initial adrenaline uptake by pigmented tissues is not explained by pigment binding. It is proposed that the increased initial binding of adrenaline by pigmented tissues is caused by a difference between pigmented and albino tissues in the number of adrenergic neurones; this is also supported by the literature. (author)

Scanning electron microscopy and recording of the physiological activity of tracheal ciliated cells treated by fractionated irradiation

International Nuclear Information System (INIS)

Albertsson, M.; Hakansson, C.H.; Mecklenburg, C. von

1983-01-01

The ciliated epithelium of the rabbit's trachea was irradiated with daily fractions of 2 Gy to an accumulated dose of 20 Gy. The beat frequency of the cilia was recorded and specimens were taken for SEM-, TEM- and LM-investigations. Examination was made 1-10 days after each fractionation schedule. An increased ciliary beat frequency was recorded at 2 Gy and 4 Gy. With increasing dose, there was an inverted relationship to the frequency. Light-microscopy showed edema and an increased amount of goblet cells in relation to the increasing dose. With SEM an increased number of ciliary blebs could be seen. These could be classified according to size and number, and showed a positive correlation to the dose. TEM-investigations showed signs of increased intracellular activity at higher doses in the form of multilobulated nuclei and an increasing number of nuclear pores. At lower doses, an increased amount of mitochondria appeared in the apical part of the cell. It is at present difficult to evaluate any correlation between the physiological activity and the morphology. More biological data are needed to explain the early irradiation effects

[Two cases of Vogt-Koyanagi-Harada disease presenting shallow anterior chamber].

Science.gov (United States)

Takemoto, Daisuke; Ijiri, Shigeyuki; Shimizu, Michiharu; Higashide, Tomomi; Sugiyama, Kazuhisa

2015-05-01

We report two cases of Vogt-Koyanagi-Harada disease (VKH) in which shallow anterior chambers were improved after steroid pulse therapy. The patients were women aged 65 and 72. They had headaches, decreased visual acuity and shallow anterior chamber in both eyes. There was no inflammation in the anterior chamber. Ultrasound biomicroscopy (UBM) showed ciliary edema, ciliochoroidal detachment, and angle closure. One case showed high intraocular pressure (IOP), and a diagnosis of acute primary angle closure was made. Although cataract surgery was performed in the left eye, postoperative optical coherence tomography (OCT) revealed serous retinal detachment in both eyes. The shallow anterior chamber and UBM findings were improved and serous retinal detachment disappeared after steroid pulse therapy in both cases. VKH may cause shallow anterior chamber and angle closure. The inflammatory changes of VKH in the anterior segment, i. e. ciliary edema and ciliochoroidal detachment, may exacerbate the shallow anterior chambers and narrow angles and result in an acute increase in IOP in eyes with short axial length. VKH associated with shallow anterior chamber may be misdiagnosed as acute primary angle closure. For differential diagnosis, examinations of the ocular fundus including OCT are useful.

Motivated Reasoning, Political Information, and Information Literacy Education

Science.gov (United States)

Lenker, Mark

2016-01-01

Research in psychology and political science has identified motivated reasoning as a set of biases that inhibit a person's ability to process political information objectively. This research has important implications for the information literacy movement's aims of fostering lifelong learning and informed citizenship. This essay argues that…

Information Theory for Information Science: Antecedents, Philosophy, and Applications

Science.gov (United States)

Losee, Robert M.

2017-01-01

This paper provides an historical overview of the theoretical antecedents leading to information theory, specifically those useful for understanding and teaching information science and systems. Information may be discussed in a philosophical manner and at the same time be measureable. This notion of information can thus be the subject of…

Information seeking behaviour and use of information resources, by ...

African Journals Online (AJOL)

Provision of information materials to meet the information needs of students, Creation of awareness programme that will enlighten the students in getting adequate information needed in the library such as asking a librarian, the use of Online public access(OPAC) and E-granary to get the adequate information they need, ...

Informal Taxation.

Science.gov (United States)

Olken, Benjamin A; Singhal, Monica

2011-10-01

Informal payments are a frequently overlooked source of local public finance in developing countries. We use microdata from ten countries to establish stylized facts on the magnitude, form, and distributional implications of this "informal taxation." Informal taxation is widespread, particularly in rural areas, with substantial in-kind labor payments. The wealthy pay more, but pay less in percentage terms, and informal taxes are more regressive than formal taxes. Failing to include informal taxation underestimates household tax burdens and revenue decentralization in developing countries. We discuss various explanations for and implications of these observed stylized facts.

3 CFR - Classified Information and Controlled Unclassified Information

Science.gov (United States)

2010-01-01

... on Transparency and Open Government and on the Freedom of Information Act, my Administration is... memoranda of January 21, 2009, on Transparency and Open Government and on the Freedom of Information Act; (B... 3 The President 1 2010-01-01 2010-01-01 false Classified Information and Controlled Unclassified...

Disabilities Information Flow: A Disabilities Information Management System

Science.gov (United States)

Ling, Bin; Allison, Colin; Nicholl, J. Ross; Moodley, Luke; Roberts, Dave

2006-01-01

The Disabilities Information Flow (DIF) project at the University of St Andrews has sought to provide a means of efficiently managing all student disabilities information within the institution and provide appropriate role-based service interfaces for all staff who need to routinely interact with this information. This paper describes the software…

Creativity, information, and consciousness: The information dynamics of thinking.

Science.gov (United States)

Wiggins, Geraint A

2018-05-07

This paper presents a theory of the basic operation of mind, Information Dynamics of Thinking, which is intended for computational implementation and thence empirical testing. It is based on the information theory of Shannon, and treats the mind/brain as an information processing organ that aims to be information-efficient, in that it predicts its world, so as to use information efficiently, and regularly re-represents it, so as to store information efficiently. The theory is presented in context of a background review of various research areas that impinge upon its development. Consequences of the theory and testable hypotheses arising from it are discussed. Copyright © 2018. Published by Elsevier B.V.

Network Centric Information Structure - Crisis Information Management

National Research Council Canada - National Science Library

Aarholt, Eldar; Berg, Olav

2004-01-01

This paper presents a generic Network Centric Information Structure (NCIS) that can be used by civilian, military and public sectors, and that supports information handling applied to crises management and emergency response...

Promoting Information Literacy by Promoting Health Literacy in the Information Society

Directory of Open Access Journals (Sweden)

Meisam Dastani

2016-09-01

Full Text Available In the information society, the production, distribution and use of information are freely and widely available for all issues of life. Proper and appropriate use of reliable information is especially important in health care. The present study introduces the concepts and benefits of health literacy and information literacy and its role in improving health literacy. This study was a review based on the concepts of information society, information literacy and information education to present importance of promoting information literacy on health literacy in the information society. The information society is presented by providing a platform of information technology and computer systems to attempt to exchange and develop information among people in the community. Currently, electronic and web-based health information in the mass form is available. Information as a fundamental base of the information society is a phenomenon that our decisions are affected in relation to various issues such as safety and health issues. It is important to avoid the mass of invalid, incorrect and inappropriate information which is available on the internet. This requires information literacy skills such as identifying, accessing and evaluating information. In general, it can be said that the promotion of health literacy in communities requires learning different skills in the form of information literacy.Data obtained from this study can be used in developing the long term health programs to prevention of non-communicable diseases in our country

Informal Taxation*

Science.gov (United States)

Olken, Benjamin A.; Singhal, Monica

2011-01-01

Informal payments are a frequently overlooked source of local public finance in developing countries. We use microdata from ten countries to establish stylized facts on the magnitude, form, and distributional implications of this “informal taxation.” Informal taxation is widespread, particularly in rural areas, with substantial in-kind labor payments. The wealthy pay more, but pay less in percentage terms, and informal taxes are more regressive than formal taxes. Failing to include informal taxation underestimates household tax burdens and revenue decentralization in developing countries. We discuss various explanations for and implications of these observed stylized facts. PMID:22199993

Informed consent - Providing information about prenatal examinations

DEFF Research Database (Denmark)

Dahl, Katja; Kesmodel, Ulrik; Hvidman, Lone

as well.The review is based on systematic search strategy in the electronic databases Medline and Science Citation. Additional studies were identified through reference lists of individual papers obtained. Improving knowledge scores and reducing decisional conflict can be obtained by group counselling...... pregnant women about prenatal examinations. Women's knowledge, decisional conflict, satisfaction and anxiety will be explored as compared with different ways and different groups of health professionals providing information. To what extent information empowers informed decision making will be explored...

Information delivery manuals to integrate building product information into design

DEFF Research Database (Denmark)

Berard, Ole Bengt; Karlshøj, Jan

2013-01-01

Despite continuing BIM progress, professionals in the AEC industry often lack the information they need to perform their work. Although this problem could be alleviated by information systems similar to those in other industries, companies struggle to model processes and information needs....... Traditional business process modeling languages often fail to completely cover all four perspectives. BuildingSMART has proposed Information Delivery Manuals (IDMs) to model and re-engineer processes that address the four perspectives through a collaborative methodology in order to standardize and implement...... in the manner necessary to develop information systems that support digital collaboration, workflows, and information exchange. Processes for information systems can be described from four perspectives: task sequence, information need, organizational interaction, and required logic for the specific task...

Radiation information and informed consent for clinical trials

Energy Technology Data Exchange (ETDEWEB)

Caon, Martin [School of Nursing and Midwifery, Flinders University, Adelaide (Australia)], E-mail: martin.caon@flinders.edu.au

2008-09-01

Examples of the statements about the radiation from medical imaging in the information for participants provided to the Human Research Ethics Committee (HREC) for approval are presented and discussed. There is considerable scope for improvement in the information about radiation that is presented to potential participants in clinical trials. Many radiation statements seem only intended to allay fear and anxiety about radiation rather than providing accurate information. This situation cannot be said to be conducive to allowing the participant to give informed consent to their involvement in a clinical trial in which ionising radiation is used. As many clinical trials are international and conducted at many sites (sometimes over 100), we would expect the same statements to have been seen by members of HRECs in many countries. Few HRECs include a member who is an expert in radiation. Hence, to ensure that the information is sound, those sections of the participant information that refer to radiation should be written or reviewed by a specialist in radiation protection such as a medical physicist, a health physicist or a radiation safety officer. (opinion)

Radiation information and informed consent for clinical trials

International Nuclear Information System (INIS)

Caon, Martin

2008-01-01

Examples of the statements about the radiation from medical imaging in the information for participants provided to the Human Research Ethics Committee (HREC) for approval are presented and discussed. There is considerable scope for improvement in the information about radiation that is presented to potential participants in clinical trials. Many radiation statements seem only intended to allay fear and anxiety about radiation rather than providing accurate information. This situation cannot be said to be conducive to allowing the participant to give informed consent to their involvement in a clinical trial in which ionising radiation is used. As many clinical trials are international and conducted at many sites (sometimes over 100), we would expect the same statements to have been seen by members of HRECs in many countries. Few HRECs include a member who is an expert in radiation. Hence, to ensure that the information is sound, those sections of the participant information that refer to radiation should be written or reviewed by a specialist in radiation protection such as a medical physicist, a health physicist or a radiation safety officer. (opinion)

Information Diffusion in Facebook-Like Social Networks Under Information Overload

Science.gov (United States)

Li, Pei; Xing, Kai; Wang, Dapeng; Zhang, Xin; Wang, Hui

2013-07-01

Research on social networks has received remarkable attention, since many people use social networks to broadcast information and stay connected with their friends. However, due to the information overload in social networks, it becomes increasingly difficult for users to find useful information. This paper takes Facebook-like social networks into account, and models the process of information diffusion under information overload. The term view scope is introduced to model the user information-processing capability under information overload, and the average number of times a message appears in view scopes after it is generated is proposed to characterize the information diffusion efficiency. Through theoretical analysis, we find that factors such as network structure and view scope number have no impact on the information diffusion efficiency, which is a surprising result. To verify the results, we conduct simulations and provide the simulation results, which are consistent with the theoretical analysis results perfectly.

Notes à La Flore de Corse, XXV

OpenAIRE

Jeanmonod, Daniel

2015-01-01

These “notes” deal with 110 noteworthy taxa amongst which 21 are new to the island's flora. One of these taxa is native: Reseda alba subsp. hookeri and 20 are introduced: Abies pinsapo, Acer ×coriaceum, Albizia julibrissin, Arundo micrantha, Azolla filiculoides, Bolboschoenus laticarpus, Digitaria ciliaris, Digitaria violascens, Eucalyptus camaldulensis, E. globulus, Genista januensis subsp. lydia, Hedera helix subsp. maroccana, Morus kagayamae, Oenothera rosea, Paspalum vaginatum, Puccinelli...

The Effect of Climate and Spittlebug (aeneolamia albofasciata) on Buffelgrass (cenchrus cilaris L.) Productivity in the Sonoran Desert

OpenAIRE

Martin-Rivera, Martha H.

1994-01-01

I conducted field studies during 1984-1988 to determine how (1) precipitation amount and distribution affect buffelgrass [Cenchrus ciliaris L. (Link) l productivity, (2) summer rainfall amount and distribution and temperature influenced the spittlebug (Aeneolamia albofasciata Lalleman) life cycle, and (3) summer burning affects spittlebug densities and buffelgrass productivity. Experiment I was conducted from 1985 through 1988. Forage samples collected at 15 -day intervals were separated i...

G-Protein Gαs controls medulloblastoma initiation by suppressing sonic hedgehog signaling.

Science.gov (United States)

He, Xuelian; Lu, Q Richard

2015-01-01

We identify Gαs as a novel tumor suppressor in medulloblastoma that functions principally by inhibition of sonic hedgehog signaling. Gαs not only stimulates cyclic adenosine monophosphate (cAMP)-dependent signaling but also inhibits ciliary trafficking of hedgehog components. Elevation of cAMP inhibits medulloblastoma growth and augments inhibition of smoothened to decrease tumor cell proliferation, thus highlighting Gαs as a potential therapeutic target.

INFORMATION ASSURANCE - INTELLIGENCE - INFORMATION SUPERIORITY RELATIONSHIP WITHIN NATO OPERATIONS

Directory of Open Access Journals (Sweden)

Gheorghe BOARU, Ioan-Mihai ILIEŞ

2011-01-01

Full Text Available There is a tight relationship between information assurance, the intelligence cycle and information superiority within NATO operations. The intelligence cycle has a discrete architecture and provides on-time and relevant intelligence products to the joint force commanders and to other authorized users in a specifi c joint area of operations. The intelligence cycle must follow the evolution of the operation. A permanent intelligence estimate will be performed during the military decision making process and operations execution. Information superiority is one of the most powerful intelligence cycle achievements. and decisively infuences the success of NATO joint operations. Information superiority must be preserved and enhanced through information assurance. Information assurance is an information operation that must be planned by the military in charge of operation security or by non-military experts, executed by all personnel during the entire intelligence cycle life time and employed during the planning and execution of NATO joint operations.

FINANCIAL INFORMATION, EFFECTS OF FINANCIAL INFORMATION ON ECONOMIC DECISION

Directory of Open Access Journals (Sweden)

TAK ISA

2010-12-01

Full Text Available Financial information has, indisputably, an important effect in economics. To form an effective capital market, financial information must be reliable and accurate. Misleading financial information always has a negative impact on economic decision taken by users. It is known that financial information as the cornerstone of financial markets, can improve economic performance in several ways. Nowadays we are facing economic crisis due to irregularities of presentation of financial statements to users. Misunderstandings cause economic recession. Detection of fraudulent financial information, is an important issue facing the auditing profession. Currently, bankruptcy of companies around the world, leaves millions of people without jobs, this is caused by financial information which is manipulated by companies. The purpose of this paper is to analyze the effects of errors and manipulation committed in the financial information sector on the real economy. Also one of the purposes of this paper is to analyze error and fraud in financial statements how it effects the real economy and the reasons for committing fraud in financial statements. Also, several suggestions are included in this study about actions that can be taken to prevent errors and manipulation in financial information.

Relationship between stakeholders' information value perception and information security behaviour

Science.gov (United States)

Tajuddin, Sharul; Olphert, Wendy; Doherty, Neil

2015-02-01

The study, reported in this paper, aims to explore the relationship between the stakeholders' perceptions about the value of information and their resultant information security behaviours. Moreover, this study seeks to explore the role of national and organisational culture in facilitating information value assignment. Information Security is a concept that formed from the recognition that information is valuable and that there is a need to protect it. The ISO 27002 defines information as an asset, which, like other important business assets, is essential to an organisation's business and consequently needs to be appropriately protected. By definition, an asset has a value to the organisation hence it requires protection. Information protection is typically accomplished through the implementation of countermeasures against the threats and vulnerabilities of information security, for example, implementation of technological processes and mechanisms such as firewall and authorization and authentication systems, set-up of deterrence procedures such as password control and enforcement of organisational policy on information handling procedures. However, evidence routinely shows that despite such measures, information security breaches and incidents are on the rise. These breaches lead to loss of information, personal records, or other data, with consequent implications for the value of the information asset. A number of studies have suggested that such problems are not related primarily to technology problems or procedural deficiencies, but rather to stakeholders' poor compliance with the security measures that are in place. Research indicates that compliance behaviour is affected by many variables including perceived costs and benefits, national and organisational culture and norms. However, there has been little research to understand the concept of information value from the perspective of those who interact with the data, and the consequences for information

HEALTH INFORMATION TECHNOLOGY IN EXCHANGE OF HEALTH INFORMATION

Directory of Open Access Journals (Sweden)

Jordan Deliversky

2016-06-01

Full Text Available Health information technology involves the exchange of health information in an electronic environment. Data protection is comprised of many elements, including where the data resides, how it is used, and who has access to it. Individually identifiable health information should be protected with reasonable administrative, technical, and physical safeguards to ensure its confidentiality, integrity, and availability and to prevent unauthorized access, use, or disclosure. Health records are among the most sensitive records available containing information concerning an individual. The unauthorized disclosure of a medical condition or diagnosis could negatively impact an individual’s personal and professional life.

Um caso raro de discinesia ciliar primária associada a heterotaxia

Directory of Open Access Journals (Sweden)

Cátia Quintela

2009-01-01

Full Text Available Resumo: A discinesia ciliar primária é uma doença autossómica recessiva caracterizada pela história de infecções de repetição do aparelho respiratório superior e inferior, rinossinusite e bronquite associada a situs inversus completo ou parcial. Os autores apresentam um doente de 78 anos, eurocaucasiano, com rinossinusites, bronquite crónica e dispneia, otite média com défices auditivos, infertilidade, seguido em consulta de gastrenterologia por dispepsia e obstipação há vários anos. Realizou vários exames que mostraram: agenesia frontal direita, espessamento brônquico, bronquiectasias, cego e cólon ascendente localizados na fossa ilíaca esquerda. Excluiu-se imunodeficiência, alergias, fibrose quística e outros. No decurso da investigação concluímos que se tratava de um caso de discinesia ciliar primária. Pela raridade deste caso, decidimos apresentá-lo.Rev Port Pneumol 2009; XV (1: 115-120 Abstract: Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78-year-old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it.Rev Port Pneumol 2009; XV (1: 115-120 Palavras-chave: Discinesia ciliar primária, heterotaxia, Key-words: Primary ciliary dyskinesia, heterotaxy

Um caso raro de discinesia ciliar primária associada a heterotaxia

Directory of Open Access Journals (Sweden)

Cátia Quintela

2009-01-01

Full Text Available Resumo: A discinesia ciliar primária é uma doença autossómica recessiva caracterizada pela história de infecções de repetição do aparelho respiratório superior e inferior, rinossinusite e bronquite associada a situs inversus completo ou parcial. Os autores apresentam um doen te de 78 anos, eurocaucasiano, com rinossinusites, bronquite crónica e dispneia, otite média com défices auditivos, infertilidade, seguido em consulta de gastrenterologia por dispepsia e obstipação há vários anos. Realizou vários exames que mostraram: agenesia frontal direita, espessamento brônquico, bronquiectasias, cego e cólon ascendente localizados na fossa ilíaca esquerda. Excluiu-se imunodeficiência, alergias, fibrose quística e outros. No decurso da investigação concluímos que se tratava de um caso de discinesia ciliar primária. Pela raridade deste caso, decidimos apresentá-lo. Abstract: Primary ciliary dyskinesia is an autosomal recessive disease with a clinical history of upper and lowers respiratory infections, rhinosinusitis and bronquitis associated with complete or partial situs inversus. The authors present a 78-year-old male caucasian patient with rhinosinusitis, lower respiratory tract infection and dyspnea, chronic otitis with hearing deficit and infertility followed in Gastroenterology for dyspepsia and constipation. The radiological studies revealed agenesis of right frontal sinus; bronchial wall thickening; bronchiectasis; cecum and ascending colon located on the left and small bowel occupies right side of abdomen. He had no immunodeficiency, allergies, cystic fibrosis and others. We concluded primary ciliary dyskinesia with heterotaxy. For the rarity of this case we decided to present it. Palavras-chave: Discinesia ciliar primária, heterotaxia, Key-words: Primary ciliary dyskinesia, heterotaxy

Ocular pharmacokinetics of 0.45% ketorolac tromethamine.

Science.gov (United States)

Attar, Mayssa; Schiffman, Rhett; Borbridge, Lisa; Farnes, Quinn; Welty, Devin

2010-12-01

A new carboxymethylcellulose (CMC)-containing ophthalmic formulation of 0.45% ketorolac, pH 6.8 (Acuvail(®)) was recently developed for treatment of inflammation and pain after cataract surgery. This study compared pharmacokinetics of the new formulation with that of a prior formulation, 0.4% ketorolac, pH 7.4 (Acular LS(®)). Ketorolac formulations were administered bilaterally (35 μL) to female New Zealand White rabbits. Samples from aqueous humor and iris-ciliary body were collected at multiple time points, and ketorolac was quantified using liquid chromatography-tandem mass spectrometry. In aqueous humor, the peak concentration (C(max)) and area under the concentration-time curve (AUC(0-τ)) of ketorolac were, respectively, 389 ng/mL and 939 ng·h/mL following administration of the CMC-containing 0.45% ketorolac, pH 6.8, and 211 ng/mL and 465 ng·hr/mL following administration of the 0.4% ketorolac, pH 7.4. In iris-ciliary body, C(max) and AUC(0-τ) of ketorolac were, respectively 450 ng/g and 2040 ng·h/g after administration of the CMC-containing 0.45% ketorolac, pH 6.8, and 216 ng/g and 699 ng·h/g after administration of the 0.4% ketorolac, pH 7.4. PK simulations predicted an AUC(0-τ) of 2910 ng·h/g for twice daily, CMC-containing 0.45% ketorolac, pH 6.8, compared to 725 ng·h/g for 4 times daily, 0.4% ketorolac, pH 7.4. The CMC-containing formulation of 0.45% ketorolac, pH 6.8, increased ketorolac bioavailability by 2-fold in aqueous humor and by 3-fold in iris-ciliary body in comparison to the 0.4% ketorolac, pH 7.4, allowing a reduced dosing schedule from 4 times daily to twice daily.

Study on imaging diagnosis of the ciliochoroidal detachment

International Nuclear Information System (INIS)

Shi Dapeng; Feng Gansheng; Li Shuyin

2008-01-01

Objective: To investigate the characteristics of imaging appearances and diagnostic value of ciliochoroidal detachment. Methods: Ultrasound, MRI and CT appearances of 19 eases of ciliochoroidal detachment were reviewed, which included 9 cases of serous ciliochoroidal detachment, 7 cases of choroid hematoma, and 3 cases of ciliary, body detachment. All cases were examined by both B ultrasound and MRI at the same time, 11 of them were examined by CT. Results: Ultrasound showed membrane bulge of the eyeball wall with different extent in 9 cases of serous ciliochoroidal detachment. Its postzone connected the equator or close to optic disc and its prozone could not been detected by ultrasound. There was echofree fluid darkspace under detached membrane. MRI showed membrane detachment at both nasal side and temporal side of ocular annelation in 9 cases of ciliochoroidal detachment. The detached membrane was approximately symmetry, its anterior margin did not exceed ciliary body adhesion of ocular annelation and its posterior margin stopped at posterior part of ocular annelation. There was long T 1 and long T 2 signal under detached membrane. CT showed slight thickening of ocular annelation and slightly increased density of eyeball in 5 eases of serous ciliochoroidal detachment. Ultrasound showed hemispheroid or curve bulge of eyeball wall, projected toward the vitreous cavity in 7 cases of choroid hematoma, and there was an amount of low echo light spot under the detached membrane. Both MRI (7 cases) and CT (5 cases) showed hemispheroid, fusiform or curve bulge on the nasal side, temporal side and posterior part of ocular annelation. They appeared as isodensity or slight high density on CT, iso- or high signal on T 1 WI and T 2 WI. Stratification was seen on T 2 WI in 1 case of choroid hematoma. Ciliary body detachment could not been detected by either MRI (3 cases) or CT (1 case) except ultrasound. Conclusion: Imaging examination may provide reliable diagnostic evidence

Smart use of data, information and communication: the INFORM-ed Best Local Practice Project--Grafton Base Hospital.

Science.gov (United States)

Lloyd, Sheree; Collie, Jean; McInnes, Alastair; King, Kevin; Lollback, Alison; Garland, Angie

This paper describes current progress for an information management project in a medium-sized rural hospital after the first four months of the one-year project. In particular, the article examines some of the project outcomes to date as these relate to the National Hospitals and Health Reform recommendations for the smart use of data, information and communication. The paper identifies a number of important challenges and issues that have been addressed by the project and proposes that the project findings may be used to inform similar projects in other settings. These findings relate to clinician requirements for reports, investment in human resources, development, and time for information management activities. An understanding of data collected, information systems, and presentation of clinician data are also important. The benefits of information sharing in assisting quality improvement activities are particularly relevant but, more importantly, they can engage and involve clinicians in the use of information. The importance of local data, information, and knowledge is described. Finally, issues for the health information management profession, such as working collegially and sharing knowledge and expertise, are outlined.

Information logistics: A production-line approach to information services

Science.gov (United States)

Adams, Dennis; Lee, Chee-Seng

1991-01-01

Logistics can be defined as the process of strategically managing the acquisition, movement, and storage of materials, parts, and finished inventory (and the related information flow) through the organization and its marketing channels in a cost effective manner. It is concerned with delivering the right product to the right customer in the right place at the right time. The logistics function is composed of inventory management, facilities management, communications unitization, transportation, materials management, and production scheduling. The relationship between logistics and information systems is clear. Systems such as Electronic Data Interchange (EDI), Point of Sale (POS) systems, and Just in Time (JIT) inventory management systems are important elements in the management of product development and delivery. With improved access to market demand figures, logisticians can decrease inventory sizes and better service customer demand. However, without accurate, timely information, little, if any, of this would be feasible in today's global markets. Information systems specialists can learn from logisticians. In a manner similar to logistics management, information logistics is concerned with the delivery of the right data, to the ring customer, at the right time. As such, information systems are integral components of the information logistics system charged with providing customers with accurate, timely, cost-effective, and useful information. Information logistics is a management style and is composed of elements similar to those associated with the traditional logistics activity: inventory management (data resource management), facilities management (distributed, centralized and decentralized information systems), communications (participative design and joint application development methodologies), unitization (input/output system design, i.e., packaging or formatting of the information), transportations (voice, data, image, and video communication systems

Information Security Service Branding – beyond information security awareness

Directory of Open Access Journals (Sweden)

Rahul Rastogi

2012-12-01

Full Text Available End-users play a critical role in the effective implementation and running of an information security program in any organization. The success of such a program depends primarily on the effective implementation and execution of associated information security policies and controls and the resultant behavior and actions of end-users. However, end-users often have negative perception of information security in the organization and exhibit non-compliance. In order to improve compliance levels, it is vital to improve the image of information security in the minds of end-users. This paper borrows the concepts of brands and branding from the domain of marketing to achieve this objective and applies these concepts to information security. The paper also describes a process for creating the information security service brand in the organization.

Core information sets for informed consent to surgical interventions: baseline information of importance to patients and clinicians.

Science.gov (United States)

Main, Barry G; McNair, Angus G K; Huxtable, Richard; Donovan, Jenny L; Thomas, Steven J; Kinnersley, Paul; Blazeby, Jane M

2017-04-26

Consent remains a crucial, yet challenging, cornerstone of clinical practice. The ethical, legal and professional understandings of this construct have evolved away from a doctor-centred act to a patient-centred process that encompasses the patient's values, beliefs and goals. This alignment of consent with the philosophy of shared decision-making was affirmed in a recent high-profile Supreme Court ruling in England. The communication of information is central to this model of health care delivery but it can be difficult for doctors to gauge the information needs of the individual patient. The aim of this paper is to describe 'core information sets' which are defined as a minimum set of consensus-derived information about a given procedure to be discussed with all patients. Importantly, they are intended to catalyse discussion of subjective importance to individuals. The model described in this paper applies health services research and Delphi consensus-building methods to an idea orginally proposed 30 years ago. The hypothesis is that, first, large amounts of potentially-important information are distilled down to discrete information domains. These are then, secondly, rated by key stakeholders in multiple iterations, so that core information of agreed importance can be defined. We argue that this scientific approach is key to identifying information important to all stakeholders, which may otherwise be communicated poorly or omitted from discussions entirely. Our methods apply systematic review, qualitative, survey and consensus-building techniques to define this 'core information'. We propose that such information addresses the 'reasonable patient' standard for information disclosure but, more importantly, can serve as a spring board for high-value discussion of importance to the individual patient. The application of established research methods can define information of core importance to informed consent. Further work will establish how best to incorporate

78 FR 18593 - Information Collection; Information Regarding Responsibility Matters

Science.gov (United States)

2013-03-27

... developed to address these requirements. FAPIIS provides users access to integrity and performance..., company name (if any), and ``Information Collection 9000-0174, Information Regarding Responsibility... accuracy and completeness, their integrity and performance information upon which responsibility...

Information Needs and Source Information of Agricultural Extension Workers in DIY

Directory of Open Access Journals (Sweden)

Retno Wulandari

2016-02-01

Full Text Available One of the important factors that contribute to agricultural development is information. As such agricultural extension workers that link agricultural agencies to the farmers must have adequate and up-to-date information about agricultural practices before they could inform, educate and train the farmers in method and techniques of farming. This study aims to determine the types of information needed by agricultural extension workers; to determine the sources of information used by the agricultural extension workers; and to determine the comparison between types of information with information sources used by agricultural extension workers. The present study was a descriptive study using a survey design and conducted at Daerah Istimewa Yogyakarta (DIY Province. The populations of the study consists 181 agricultural extension workers divisible in the district level, namely Sleman, Kulon Progo, Gunung Kidul dan Bantul. Sampling procedure in this study used cluster sampling. The method for gathering data from the respondents is based on a structured questionnaire. Descriptive statistics was to describe the characteristics of the respondents and the dependent variables of the study. As a result, most of agricultural extension workers strongly needed the innovation information in seeking information followed by extension service information and extension role information. The result of information sources used by agricultural extension worker for extension service information, innovation information and extension role information indicates that agricultural extension workers used interpersonal source. Most of them never sought information use Internet, they use radio more frequently than television and video, agricultural extension worker also used mass media as information source.

Information Impact: Journal of Information and Knowledge ...

African Journals Online (AJOL)

Information Impact: Journal of Information and Knowledge Management. Journal Home · ABOUT THIS JOURNAL · Advanced Search · Current Issue · Archives · Journal Home > Vol 7, No 2 (2016) >. Log in or Register to get access to full text downloads.

Information Literacy

African Journals Online (AJOL)

ian

The paper also discusses some challenges faced in the teaching ... groups, language and cultural barriers and time and lack of computers. The paper ... administrative staff, researchers and librarians work with information. ...... The role of Information and Communication Technologies in Harnessing Information for women in.

How social information affects information search and choice in probabilistic inferences.

Science.gov (United States)

Puskaric, Marin; von Helversen, Bettina; Rieskamp, Jörg

2018-01-01

When making decisions, people are often exposed to relevant information stemming from qualitatively different sources. For instance, when making a choice between two alternatives people can rely on the advice of other people (i.e., social information) or search for factual information about the alternatives (i.e., non-social information). Prior research in categorization has shown that social information is given special attention when both social and non-social information is available, even when the social information has no additional informational value. The goal of the current work is to investigate whether framing information as social or non-social also influences information search and choice in probabilistic inferences. In a first study, we found that framing cues (i.e., the information used to make a decision) with medium validity as social increased the probability that they were searched for compared to a task where the same cues were framed as non-social information, but did not change the strategy people relied on. A second and a third study showed that framing a cue with high validity as social information facilitated learning to rely on a non-compensatory decision strategy. Overall, the results suggest that social in comparison to non-social information is given more attention and is learned faster than non-social information. Copyright © 2017 Elsevier B.V. All rights reserved.

78 FR 73819 - Information Collection; Financial Information Security Request Form

Science.gov (United States)

2013-12-09

... DEPARTMENT OF AGRICULTURE Forest Service Information Collection; Financial Information Security..., Financial Information Security Request Form. DATES: Comments must be received in writing on or before... Information Security Request Form. OMB Number: 0596-0204. Expiration Date of Approval: 02/28/2014. Type of...

Information Science Research: The Search for the Nature of Information.

Science.gov (United States)

Kochen, Manfred

1984-01-01

High-level scientific research in the information sciences is illustrated by sampling of recent discoveries involving adaptive information processing strategies, computer and information systems, centroid scaling, economic growth of computer and communication industries, and information flow in biological systems. Relationship of information…

Collaborative information seeking

DEFF Research Database (Denmark)

Hertzum, Morten

2008-01-01

Since common ground is pivotal to collaboration, this paper proposes to define collaborative information seeking as the combined activity of information seeking and collaborative grounding. While information-seeking activities are necessary for collaborating actors to acquire new information......, the activities involved in information seeking are often performed by varying subgroups of actors. Consequently, collaborative grounding is necessary to share information among collaborating actors and, thereby, establish and maintain the common ground necessary for their collaborative work. By focusing...... on the collaborative level, collaborative information seeking aims to avoid both individual reductionism and group reductionism, while at the same time recognizing that only some information and understanding need be shared....

Information Impact | Journal of Information and Knowledge ...

African Journals Online (AJOL)

USER

Information Impact | Journal of Information and Knowledge Management ... Abstract. This study was designed to determine the impact of collaboration on research and teaching .... delivery of quality instruction. ... for doing Collaborative projects, and there ... reveals that co-construction of knowledge ..... Get relevant materials.

76 FR 78009 - Information Collection; Implementation of Information Technology Security Provision

Science.gov (United States)

2011-12-15

...] Information Collection; Implementation of Information Technology Security Provision AGENCY: General Services... collection requirement regarding Implementation of Information Technology Security Provision. Public comments... Information Collection 3090- 0294, Implementation of Information Technology Security Provision, by any of the...

Information Literacy Practices and Student Protests: Mapping Community Information Landscapes

Science.gov (United States)

Špiranec, Sonja; Kos, Denis

2013-01-01

Introduction: This paper provides a contribution to understandings of information literacy regarding context and transferability of information practices. Specifically, the paper analyses the subset of information practices in situations of student protests and addresses issues of transfer of information literacy practice from a highly formal…

Towards Information Lasers

Directory of Open Access Journals (Sweden)

Andrei Khrennikov

2015-10-01

Full Text Available Recently, the methods of quantum theory (QT, especially quantum information and probability, started to be widely applied outside of physics: in cognitive, social and political sciences, psychology, economics, finances, decision making, molecular biology and genetics. Such models can be called quantum-like, in contrast to real quantum physical cognitive and biological models. Quantum-like means that only the information and probability structures of QT are explored. This approach matches the information interpretation of QT well (e.g., Zeilinger and Brukner, Fuchs and Mermin, D’Ariano, as well as the informational viewpoint on physics in general (e.g., Wheeler’s “it from bit” paradigm. In this paper, we propose a quantum-like model of an information laser by precessing the assumptions on the structure of state spaces of information processors, “information atoms” (i-atoms and information fields. The basic assumption is the discrete structure of state spaces related to quantization of an information analog of energy. To analyze a possible structure of the state space of i-atoms leading to the possibility to create information lasers, we have to develop a purely information version of quantum thermodynamics. We did this by placing the main attention on the derivation of the conditions for the equilibrium of information exchange between i-atoms and a quantized information field.

Information technology to support informal knowledge sharing

NARCIS (Netherlands)

Davison, R.M.; Ou, C.X.J.; Martinsons, M.G.

2013-01-01

The knowledge management (KM) literature largely focuses on the explicit and formal representation of knowledge in computer-based KM systems. Informal KM practices are widespread, but less is known about them. This paper aims to redress this imbalance by exploring the use of interactive information

The Information "Revolution": Information, Communications and Culture.

Science.gov (United States)

Ostry, Bernard

Today's communications systems and technology facilitate the erosion of cultural differences, threatening cultural sovereignty. In the fifteenth century, the first information revolution created the concept of the nation-state with its unique cultural identity. The technology of the second information revolution, which has advanced video…

Information as Wealth.

Science.gov (United States)

Deruchie, Douglas M.

1992-01-01

Discusses the value of information-based services in today's global economy. The combination of information technology with library and information services at an international accounting and auditing firm is described; the Global Information Network is explained; and the importance of the appropriate use of information is discussed. (LRW)

Information and Innovation Management within Information Technology Enterprises

OpenAIRE

Geoff D. Skinner

2010-01-01

Australia, while being a large and eager consumer of innovative and cutting edge Information and Communication Technologies (ICT), continues to struggle to remain a leader in Technological Innovation. This paper has two main contributions to address certain aspects of this complex issue. The first being the current findings of an ongoing research project on Information and Innovation Management in the Australian Information and Communication Technologies (ICT) sector. The...

Information

International Nuclear Information System (INIS)

Boyard, Pierre.

1981-01-01

The fear for nuclear energy and more particularly for radioactive wastes is analyzed in the sociological context. Everybody agree on the information need, information is available but there is a problem for their diffusion. Reactions of the public are analyzed and journalists, scientists and teachers have a role to play [fr

Information retrieval pathways for health information exchange in multiple care settings

DEFF Research Database (Denmark)

Kierkegaard, Patrick; Kaushal, Rainu; Vest, Joshua R.

2014-01-01

Objectives To determine which health information exchange (HIE) technologies and information retrieval pathways healthcare professionals relied on to meet their information needs in the context of laboratory test results, radiological images and reports, and medication histories. Study Design...... The study reveals that healthcare professionals used a complex combination of information retrieval pathways for HIE to obtain clinical information from external organizations. The choice for each approach was setting- and information-specific, but was also highly dynamic across users and their information...... needs. Conclusions Our findings about the complex nature of information sharing in healthcare provide insights for informatics professionals about the usage of information; indicate the need for managerial support within each organization; and suggest approaches to improve systems for organizations...

A geographical information based multimedia information system development for nuclear control

International Nuclear Information System (INIS)

Kim, H. T.; Park, S. S.; Lee, J. S.; Lee, J. W.; Shin, J. S.

2000-01-01

Current information technology is centered on the internet and changes our daily working pattern, particularly with multimedia information. Rapid development of information processing hardware and software has enabled us to deploy multimedia information management system of low hit counts and small amount of information volume on the desktop computer and publish multimedia information directly to the workgroup intranet with no particular additional hardware and software. Success of the timely development of the information system depends on the adoption of the proper direction and scale of information technology. The nuclear control mainly consists of safeguards, physical protection and export/import control. This paper provides an investigation on the application of openly available multiple media information to the nuclear control information management system. Information system with spatial map, image data including satellite imagery, audio, and video makes users easy to understand the current status and communicate each other easily. The Digital Terrain Elevation Data (DTED) Level 0 of the U.S. NIMA (National Imagery and Mapping Agency) is used as a base map. The multimedia information system is mainly built with Microsoft PowerPoint 2000 and Office Web component. A database with the second normal form was applied to the Office Web component. The importance of the information security was stressed

Why (just) information is not enough: The contributions of information services in the management of healthcare information

Science.gov (United States)

Kostagiolas, P.; Lappa, E.

2015-02-01

Information is at the centre of every hospital activity including clinical decisions and healthcare service delivery systems. Although information is an important hospital asset, several issues related to its management and organization needs to be addressed within the hospitals. The management of healthcare information is a strategic goal related to the reduction of healthcare service provision costs, and to the improvement of quality and safety of healthcare services. By discussing the rather obvious necessity for information organization and management in the healthcare domain, this work aims at the role of healthcare information services, i.e. hospital libraries and patient medical records. Finally, a typology of information services' contributions to hospital environment is presented.

Why (just) information is not enough: The contributions of information services in the management of healthcare information

Energy Technology Data Exchange (ETDEWEB)

Kostagiolas, P., E-mail: pkostagiolas@ionio.gr [Assistant Professor Department of Archives, Library Science and Museology, Ionian University, CORFU 49100 (Greece); Lappa, E., E-mail: evlappa@med.uoa.gr [Director of Medical Library of General Hospital Attikis KAT, Nikis 2 str, 14564 KIFFISIA-ATHENS (Greece)

2015-02-09

Information is at the centre of every hospital activity including clinical decisions and healthcare service delivery systems. Although information is an important hospital asset, several issues related to its management and organization needs to be addressed within the hospitals. The management of healthcare information is a strategic goal related to the reduction of healthcare service provision costs, and to the improvement of quality and safety of healthcare services. By discussing the rather obvious necessity for information organization and management in the healthcare domain, this work aims at the role of healthcare information services, i.e. hospital libraries and patient medical records. Finally, a typology of information services’ contributions to hospital environment is presented.

Why (just) information is not enough: The contributions of information services in the management of healthcare information

International Nuclear Information System (INIS)

Kostagiolas, P.; Lappa, E.

2015-01-01

Information is at the centre of every hospital activity including clinical decisions and healthcare service delivery systems. Although information is an important hospital asset, several issues related to its management and organization needs to be addressed within the hospitals. The management of healthcare information is a strategic goal related to the reduction of healthcare service provision costs, and to the improvement of quality and safety of healthcare services. By discussing the rather obvious necessity for information organization and management in the healthcare domain, this work aims at the role of healthcare information services, i.e. hospital libraries and patient medical records. Finally, a typology of information services’ contributions to hospital environment is presented