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https://www.readbyqxmd.com/read/28544585/effects-of-ophthalmologic-solutions-on-sinonasal-ciliated-epithelium
#1
Alan D Workman, Ryan M Carey, Michael A Kohanski, Nithin D Adappa, James N Palmer, Noam A Cohen
BACKGROUND: Off-label use of topical ophthalmologic formulations for treatment of rhinologic disease is cited in recent literature and is anecdotally prevalent among practicing otolaryngologists. Steroids, antibiotics, and other drugs designed for ocular use have subjective clinical efficacy in the nose and sinuses, but their specific effects on the ciliated epithelium are less well defined. This study examines 9 commercially available ophthalmologic drug formulations for effects on ciliary motility in sinonasal cultures, in an effort to characterize their utility as topical therapies for sinonasal diseases...
May 23, 2017: International Forum of Allergy & Rhinology
https://www.readbyqxmd.com/read/28541963/correlation-of-structural-and-functional-outcome-measures-in-a-phase-one-trial-of-ciliary-neurotrophic-factor-in-type-2-idiopathic-macular-telangiectasia
#2
Ferenc B Sallo, Irene Leung, Traci E Clemons, Tunde Peto, Emily Y Chew, Daniel Pauleikhoff, Alan C Bird
PURPOSE: Macular telangiectasia Type 2 is a bilateral, progressive, potentially blinding retinal disease characterized by both vascular and neurodegenerative signs. Both the area of the break in the ellipsoid zone seen in "en face" optical coherence tomographic (OCT) images and microperimetric focal retinal sensitivity loss have been proposed as potential measures of progression in macular telangiectasia. The authors aimed to assess the characteristics and interrelationship of these structural and functional disease markers from the data collected in a phase one clinical trial of ciliary neurotrophic factor in macular telangiectasia...
May 23, 2017: Retina
https://www.readbyqxmd.com/read/28530676/mutations-in-dzip1l-which-encodes-a-ciliary-transition-zone-protein-cause-autosomal-recessive-polycystic-kidney-disease
#3
Hao Lu, Maria C Rondón Galeano, Elisabeth Ott, Geraldine Kaeslin, P Jaya Kausalya, Carina Kramer, Nadina Ortiz-Brüchle, Nadescha Hilger, Vicki Metzis, Milan Hiersche, Shang Yew Tay, Robert Tunningley, Shubha Vij, Andrew D Courtney, Belinda Whittle, Elke Wühl, Udo Vester, Björn Hartleben, Steffen Neuber, Valeska Frank, Melissa H Little, Daniel Epting, Peter Papathanasiou, Andrew C Perkins, Graham D Wright, Walter Hunziker, Heon Yung Gee, Edgar A Otto, Klaus Zerres, Friedhelm Hildebrandt, Sudipto Roy, Carol Wicking, Carsten Bergmann
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...
May 22, 2017: Nature Genetics
https://www.readbyqxmd.com/read/28529994/rapamycin-increases-length-and-mechanosensory-function-of-primary-cilia-in-renal-epithelial-and-vascular-endothelial-cells
#4
Rinzhin T Sherpa, Kimberly F Atkinson, Viviana P Ferreira, Surya M Nauli
Primary cilia arebiophysically-sensitive organelles responsible for sensing fluid-flow and transducing this stimulus into intracellular responses. Previous studies have shown that the primary cilia mediate flow-induced calcium influx, and sensitivity of cilia function to flow is correlated to cilia length. Cells with abnormal cilia length or function can lead to a host of diseases that are collectively termed as ciliopathies. Rapamycin, a potent inhibitor of mTOR (mammalian target of rapamycin), has been demonstrated to be a potential pharmacological agent against the aberrant mTOR signaling seen in ciliopathies such as polycystic kidney disease (PKD) and tuberous sclerosis complex (TSC)...
December 2016: Int Educ Res J
https://www.readbyqxmd.com/read/28523001/the-role-of-transient-receptor-potential-vanilloid-4-in-pulmonary-inflammatory-diseases
#5
REVIEW
Rachel G Scheraga, Brian D Southern, Lisa M Grove, Mitchell A Olman
Ion channels/pumps are essential regulators of organ homeostasis and disease. In the present review, we discuss the role of the mechanosensitive cation channel, transient receptor potential vanilloid 4 (TRPV4), in cytokine secretion and pulmonary inflammatory diseases such as asthma, cystic fibrosis (CF), and acute lung injury/acute respiratory distress syndrome (ARDS). TRPV4 has been shown to play a role in lung diseases associated with lung parenchymal stretch or stiffness. TRPV4 indirectly mediates hypotonicity-induced smooth muscle contraction and airway remodeling in asthma...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28522413/microglia-support-atf3-positive-neurons-following-hypoglossal-nerve-axotomy
#6
Tatsuhide Tanaka, Koichi Murakami, Yoshio Bando, Taichi Nomura, Ayami Isonishi, Shoko Morita-Takemura, Kouko Tatsumi, Akio Wanaka, Shigetaka Yoshida
Microglia are essential in developmental processes and maintenance of neuronal homeostasis. Experimental axotomy of motor neurons results in neurodegeneration, and microglia in motor nuclei become activated and migrate towards injured neurons. However, whether these activated microglia are protective or destructive to neurons remains controversial. In the present study, we transected the hypoglossal nerve in BALB/c mice, causing activating transcription factor 3 (ATF3) and growth associated protein 43 (GAP43) induction, and partial neuronal death...
May 15, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28520637/clinical-experience-of-stereotactic-radiosurgery-at-a-linear-accelerator-for-intraocular-melanoma
#7
Alena Furdova, Miron Sramka, Martin Chorvath, Gabriel Kralik, Robert Furda, Michal Gregus
Long-term results with linear accelerator LINAC-based stereotactic radiosurgery for intraocular uveal malignant melanoma were assessed. A retrospective study was carried out of patients with uveal melanoma after a 1-day session stereotactic radiosurgery at LINAC in Slovakia. In the period 2001-2015, a group of 150 patients with uveal melanoma (139 choroidal melanoma, 11 ciliary body melanoma) was treated. The median tumor volume at baseline was 0.5 cm (with range from 0.2 to 1.6 cm). Tumors ranged in size from 2...
May 17, 2017: Melanoma Research
https://www.readbyqxmd.com/read/28504201/cilia-the-sensory-antennae-in-the-eye
#8
REVIEW
Helen May-Simera, Kerstin Nagel-Wolfrum, Uwe Wolfrum
Cilia are hair-like projections found on almost all cells in the human body. Originally believed to function merely in motility, the function of solitary non-motile (primary) cilia was long overlooked. Recent research has demonstrated that primary cilia function as signalling hubs that sense environmental cues and are pivotal for organ development and function, tissue hoemoestasis, and maintenance of human health. Cilia share a common anatomy and their diverse functional features are achieved by evolutionarily conserved functional modules, organized into sub-compartments...
May 11, 2017: Progress in Retinal and Eye Research
https://www.readbyqxmd.com/read/28495528/hepatic-ischemia-reperfusion-injury-disrupts-the-homeostasis-of-kidney-primary-cilia-via-oxidative-stress
#9
Sang Jun Han, Hee-Seong Jang, Sung Young Seu, Hee-Jung Cho, Yoon Jin Hwang, Jee In Kim, Kwon Moo Park
Acute kidney injury (AKI) is a major complication of hepatic surgeries. The primary cilium protrudes to the lumen of kidney tubules and plays an important role in renal functions. Disruption of primary cilia homeostasis is highly associated with human diseases including AKI. Here, we investigated whether transient hepatic ischemia induces length change and deciliation of kidney primary cilia, and if so, whether reactive oxygen species (ROS)/oxidative stress regulates those. HIR induced damages to the liver and kidney with increases in ROS/oxidative stress...
May 8, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28494707/histologic-and-ultrastructural-findings-in-dogs-with-chronic-respiratory-disease-suspected-of-ciliary-dyskinesia
#10
Ileana C Miranda, Jennifer L Granick, Anibal G Armién
Mucociliary clearance is a main defense mechanism of the respiratory tract, which can be inherently impaired in primary ciliary dyskinesia (PCD) or reversibly altered in secondary ciliary dyskinesia (SCD). Limited diagnostic test availability likely leads to misdiagnosis or underdiagnosis of PCD in animals. This study evaluated the light and transmission electron microscopy (TEM) changes in the respiratory mucosa of 15 dogs with chronic respiratory disease suspected of PCD. Necropsy was performed in 1 case and 2 dogs were used as negative controls...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/28494163/expression-of-14-3-3-zeta-protein-in-dexamethasone-treated-mice-and-human-tm-1-cells
#11
Qian Liu, Yiming Ye, Xianchai Lin, Yangfan Yang, Kaili Wu, Minbin Yu
PURPOSE: 14-3-3 zeta protein plays a potential protective role in neurodegenerative disease. Given that glaucoma and neurodegenerative diseases share a similar pathogenesis, it is possible that 14-3-3 zeta may have a similar protective effect in the glaucomatous process. In the present study, we measured the expression of 14-3-3 zeta in vivo (mouse eyes) and in vitro in a transformed human trabecular meshwork (HTM) cell line, TM-1, and assessed the possible roles of this protein in dexamethasone (DEX)-treated eyes and HTM cells...
May 11, 2017: Current Eye Research
https://www.readbyqxmd.com/read/28481653/accuracy-of-nasal-nitric-oxide-measurement-as-a-diagnostic-test-for-primary-ciliary-dyskinesia-a-systematic-review-and-meta-analysis
#12
Adam J Shapiro, Maureen Josephson, Margaret Rosenfeld, Ozge Yilmaz, Stephanie D Davis, Deepika Polineni, Elena Guadagno, Margaret W Leigh, Valery Lavergne
BACKGROUND: Primary Ciliary Dyskinesia (PCD) is a rare disorder causing chronic oto-sino-pulmonary disease, generally diagnosed through evaluation of respiratory cilia ultrastructure and/or genetic testing. Nasal nitric oxide (nNO) measurement is a PCD screening test, as PCD patients have low nNO levels, but its value as a diagnostic test remains unknown. OBJECTIVE: Perform a systematic review assessing the utility of nNO measurement (index test) as a diagnostic tool compared to the reference standard of electron microscopy (EM) evaluation of ciliary defects and/or detection of biallelic mutations in PCD genes...
May 8, 2017: Annals of the American Thoracic Society
https://www.readbyqxmd.com/read/28480027/effects-of-hydrogen-rich-saline-on-endotoxin-induced-uveitis
#13
Wei-Ming Yan, Lei Zhang, Tao Chen, Guan-Hua Zhao, Pan Long, Jing An, Zuo-Ming Zhang
The therapeutic effects of hydrogen-rich saline (HRS) have been reported for a wide range of diseases mainly via selectively reducing the amount of reactive oxygen species. Oxidative stress plays an important role in the pathogenesis of uveitis and endotoxin-induced uveitis (EIU). In this study, we investigated whether HRS can mitigate EIU in rats. Sprague-Dawley rats were randomly divided into Norm group, Model group, HRS group, dexamethasone (DEX) group, and rats in the latter three groups were injected with equal amount of lipopolysaccharide (LPS) to induce EIU of different severities (by 1 mg/kg of LPS, or 1/8 mg/kg of LPS)...
January 2017: Medical Gas Research
https://www.readbyqxmd.com/read/28473847/the-radial-organization-of-neuronal-primary-cilia-is-acutely-disrupted-by-seizure-and-ischemic-brain-injury
#14
Gregory W Kirschen, Hanxiao Liu, Tracy Lang, Xuelin Liang, Shaoyu Ge, Qiaojie Xiong
BACKGROUND: Neuronal primary cilia are sensory organelles that are critically involved in the proper growth, development, and function of the central nervous system (CNS). Recent work also suggests that they signal in the context of CNS injury, and that abnormal ciliary signaling may be implicated in neurological diseases. METHODS: We quantified the distribution of neuronal primary cilia alignment throughout the normal adult mouse brain by immunohistochemical staining for the primary cilia marker adenylyl cyclase III (ACIII) and measuring the angles of primary cilia with respect to global and local coordinate planes...
April 2017: Frontiers in Biology
https://www.readbyqxmd.com/read/28466357/the-effects-of-the-stem-cell-on-ciliary-regeneration-of-injured-rabbit-sinonasal-epithelium
#15
Ali Kavuzlu, Emel Çadallı Tatar, Tuğba Karagöz, Ferda Alpaslan Pınarlı, İlkan Tatar, Ömer Bayır, Mehmet Hakan Korkmaz
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...
May 2, 2017: European Archives of Oto-rhino-laryngology
https://www.readbyqxmd.com/read/28460059/insights-into-ciliary-genes-and-evolution-from-multi-level-phylogenetic-profiling
#16
Yannis Nevers, Megana K Prasad, Laetitia Poidevin, Kirsley Chennen, Alexis Allot, Arnaud Kress, Raymond Ripp, Julie D Thompson, Hélène Dollfus, Olivier Poch, Odile Lecompte
Cilia (flagella) are important eukaryotic organelles, present in the Last Eukaryotic Common Ancestor, and are involved in cell motility and integration of extracellular signals. Ciliary dysfunction causes a class of genetic diseases, known as ciliopathies, however current knowledge of the underlying mechanisms is still limited and a better characterization of genes is needed. As cilia have been lost independently several times during evolution and they are subject to important functional variation between species, ciliary genes can be investigated through comparative genomics...
April 28, 2017: Molecular Biology and Evolution
https://www.readbyqxmd.com/read/28460050/ift81-as-a-candidate-gene-for-nonsyndromic-retinal-degeneration
#17
Rachayata Dharmat, Wei Liu, Zhongqi Ge, Zixi Sun, Lizhu Yang, Yumei Li, Keqing Wang, Kandace Thomas, Ruifang Sui, Rui Chen
Purpose: IFT81, a core component of the IFT-B complex, involved in the bidirectional transport of ciliary proteins, has been recently implicated in syndromic ciliopathies. However, none of the IFT-B core complex proteins have been associated with nonsyndromic retinal dystrophies. Given the importance of ciliary transport in photoreceptor function and structural maintenance, we sought to investigate the impact of IFT (intraflagellar transport) mutations in nonsyndromic retinopathies. Methods: Whole exome sequencing was performed on 50 cone-rod dystrophy (CRD) patients that were previously screened for mutations in known retinal disease genes...
May 1, 2017: Investigative Ophthalmology & Visual Science
https://www.readbyqxmd.com/read/28459101/innate-immunity-and-chronic-rhinosinusitis-what-we-have-learned-from-animal-models
#18
Nyall R London, Andrew P Lane
OBJECTIVE: Chronic rhinosinusitis (CRS) is a heterogeneous and multifactorial disease characterized by dysregulated inflammation. Abnormalities in innate immune function including sinonasal epithelial cell barrier function, mucociliary clearance, response to pathogen-associated molecular patterns (PAMPs) via pattern recognition receptors (PRRs), and the contribution of innate immune cells will be highlighted in this review. DATA SOURCES: PubMed literature review...
June 2016: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/28456398/effect-of-circulating-exosomes-from-transition-cows-on-madin-darby-bovine-kidney-cell-function
#19
M A Crookenden, C G Walker, H Peiris, Y Koh, F Almughlliq, K Vaswani, S Reed, A Heiser, J J Loor, J K Kay, S Meier, S S Donkin, A Murray, V S R Dukkipati, J R Roche, M D Mitchell
The greatest risk of metabolic and infectious disease in dairy cows is during the transition from pregnancy to lactating (i.e., the transition period). The objective of this experiment was to determine the effects of extracellular vesicles (microvesicles involved in cell-to-cell signaling) isolated from transition cows on target cell function. We previously identified differences in the protein profiles of exosomes isolated from cows divergent in metabolic health status. Therefore, we hypothesized that these exosomes would affect target tissues differently...
April 26, 2017: Journal of Dairy Science
https://www.readbyqxmd.com/read/28453163/malignant-ciliary-body-medulloepithelioma-with-brain-and-parotid-metastasis
#20
Yeen Fey Ho, Yueh-Ju Tsai, Shu-Ya Wu
A 12-year-old girl with malignant ciliary medulloepithelioma and parotid metastasis was treated with semi-exenteration of the orbit and external beam radiotherapy. She had brain metastasis 7 months later and was treated with salvage chemotherapy. The patient was disease free at 11 months of follow-up. In aggressive cases, exenteration combined with radiotherapy and chemotherapy are necessary for disease control. [J Pediatr Ophthalmol Strabismus. 2017;54:e18-e22].
April 28, 2017: Journal of Pediatric Ophthalmology and Strabismus
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