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https://www.readbyqxmd.com/read/29141305/-research-progress-on-rhinitis-medicamentosa
#1
Y N Wang, Z L Luan, H T Wang
Rhinitis medicamentosa (RM) refers to nonallergic inflammation in the nasal mucosa which is caused by the abuse of nasal decongestant and it often occurs in patients with allergic/nonallergic rhinitis along with nasal congestion. RM is characterized by nasal congestion based on long-term use of nasal decongestant, without rhinorrhoea or sneezing. The signs of RM include nasal swelling, thickening, loss of elasticity, and loss of sensitivity to the decongestant. The histological changes of RM are loss of nasal mucosa cilia, squamous epithelium metaplasia, edema of epithelium cell, hyperplasia of goblet cell, increased expression of epidermal growth factor receptor and infiltration of inflammatory cells, etc...
November 7, 2017: Zhonghua Er Bi Yan Hou Tou Jing Wai Ke za Zhi, Chinese Journal of Otorhinolaryngology Head and Neck Surgery
https://www.readbyqxmd.com/read/29140789/mechanism-and-dynamics-of-inpp5e-transport-into-and-inside-the-ciliary-compartment
#2
Stefanie Kristine Kösling, Eyad Kalawy Fansa, Stefano Maffini, Alfred Wittinghofer
The inositol polyphosphate 5´-phosphatase E (INPP5E) localizes to cilia. We showed that the carrier protein phosphodiesterase 6 delta subunit (PDE6δ) mediates the sorting of farnesylated INPP5E into cilia due to high affinity binding and release by Arl3 GTP. However, the dynamics of INPP5E transport into and inside the ciliary compartment are not fully understood. Here we first investigate the movement of INPP5E using live cell fluorescence microscopy and fluorescence recovery after photobleaching (FRAP) analysis...
August 28, 2017: Biological Chemistry
https://www.readbyqxmd.com/read/29135867/analysis-of-otologic-features-of-patients-with-primary-ciliary-dyskinesia
#3
Kazuhiko Takeuchi, Masako Kitano, Hiroshi Sakaida, Satoko Usui, Sawako Masuda, Satoru Ogawa, Makoto Ikejiri, Mizuho Nagao, Takao Fujisawa, Kaname Nakatani
OBJECTIVE: To evaluate otologic features of primary ciliary dyskinesia (PCD), especially eardrum features, audiometric findings, and clinical course. STUDY DESIGN: Retrospective patient review. SETTING: Tertiary referral center. PATIENTS: Fifteen patients (mean age, 16.9 years [range, 1-32 yr]; 8 males and 7 females) diagnosed with PCD at our university hospital in the last 12 years. INTERVENTION: Diagnostic...
December 2017: Otology & Neurotology
https://www.readbyqxmd.com/read/29134781/clinical-and-molecular-genetic-characterization-of-a-male-patient-with-sensenbrenner-syndrome-cranioectodermal-dysplasia-and-biallelic-wdr35-mutations
#4
Joanna Walczak-Sztulpa, Anna Wawrocka, Anna Swiader-Lesniak, Magdalena Socha, Aleksander Jamsheer, Dorota Drozdz, Anna Latos-Bielenska, Katarzyna Zachwieja
BACKGROUND: Sensenbrenner syndrome (cranioectodermal dysplasia, CED) is a very rare autosomal recessive ciliopathy first described by Judith Sensenbrenner in 1975. CED is a complex disorder characterized by craniofacial, skeletal, and ectodermal abnormalities. The clinical symptoms are variable and the CED phenotype may present intrafamilial and interfamilial differences. Sensenbrenner syndrome belongs to a group of ciliary chondrodysplasias and is a genetically heterogeneous disease...
November 14, 2017: Birth defects research
https://www.readbyqxmd.com/read/29134552/intranasal-delivery-of-%C3%AE-asarone-to-the-brain-with-lactoferrin-modified-mpeg-pla-nanoparticles-prepared-by-premix-membrane-emulsification
#5
Linmei Pan, Jing Zhou, Feng Ju, Huaxu Zhu
Alpha-asarone is a bioactive component of Acorus tatarincuii Schott with low bioavailability, which is often used for treatments of various brain diseases in clinical setting. This study was to formulate biodegradable methoxy polyethylene glycol-polylactic acid (mPEG-PLA) nanoparticles (NPs) surface-modified by lactoferrin (Lf), for delivering α-asarone into the brain following intranasal administration. Alpha-asarone NPs were prepared by premix membrane emulsification. The relative parameters were optimized by a Box-Behnken experimental design...
November 13, 2017: Drug Delivery and Translational Research
https://www.readbyqxmd.com/read/29132433/impaired-ciliogenesis-in-differentiating-human-bronchial-epithelia-exposed-to-non-cytotoxic-doses-of-multi-walled-carbon-nanotubes
#6
Ryan J Snyder, Salik Hussain, Charles J Tucker, Scott H Randell, Stavros Garantziotis
BACKGROUND: Multi-walled carbon nanotubes (MWCNTs) are engineered nanomaterials used for a variety of industrial and consumer products. Their high tensile strength, hydrophobicity, and semi-conductive properties have enabled many novel applications, increasing the possibility of accidental nanotube inhalation by either consumers or factory workers. While MWCNT inhalation has been previously shown to cause inflammation and pulmonary fibrosis at high doses, the susceptibility of differentiating bronchial epithelia to MWCNT exposure remains unexplored...
November 13, 2017: Particle and Fibre Toxicology
https://www.readbyqxmd.com/read/29130579/a-homozygous-deleterious-cdk10-mutation-in-a-patient-with-agenesis-of-corpus-callosum-retinopathy-and-deafness
#7
Vincent J Guen, Simon Edvardson, Nitay D Fraenkel, Aviva Fattal-Valevski, Chaim Jalas, Irene Anteby, Avraham Shaag, Talia Dor, David Gillis, Eitan Kerem, Jacqueline A Lees, Pierre Colas, Orly Elpeleg
The primary cilium is a key organelle in numerous physiological and developmental processes. Genetic defects in the formation of this non-motile structure, in its maintenance and function, underlie a wide array of ciliopathies in human, including craniofacial, brain and heart malformations, and retinal and hearing defects. We used exome sequencing to study the molecular basis of disease in an 11-year-old female patient who suffered from growth retardation, global developmental delay with absent speech acquisition, agenesis of corpus callosum and paucity of white matter, sensorineural deafness, retinitis pigmentosa, vertebral anomalies, patent ductus arteriosus, and facial dysmorphism reminiscent of STAR syndrome, a suspected ciliopathy...
November 12, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29129530/glutamylation-regulates-transport-specializes-function-and-sculpts-the-structure-of-cilia
#8
Robert O'Hagan, Malan Silva, Ken C Q Nguyen, Winnie Zhang, Sebastian Bellotti, Yasmin H Ramadan, David H Hall, Maureen M Barr
Ciliary microtubules (MTs) are extensively decorated with post-translational modifications (PTMs), such as glutamylation of tubulin tails. PTMs and tubulin isotype diversity act as a "tubulin code" that regulates cytoskeletal stability and the activity of MT-associated proteins such as kinesins. We previously showed that, in C. elegans cilia, the deglutamylase CCPP-1 affects ciliary ultrastructure, localization of the TRP channel PKD-2 and the kinesin-3 KLP-6, and velocity of the kinesin-2 OSM-3/KIF17, whereas a cell-specific α-tubulin isotype regulates ciliary ultrastructure, intraflagellar transport, and ciliary functions of extracellular vesicle (EV)-releasing neurons...
November 2, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/29128370/the-inhibition-of-sphingomyelin-synthase-1-activity-induces-collecting-duct-cells-to-lose-their-epithelial-phenotype
#9
Yamila Romina Brandán, Edith Del Valle Guaytima, Nicolás Octavio Favale, Lucila Gisele Pescio, Norma B Sterin-Speziale, María Gabriela Márquez
Epithelial tissue requires that cells attach to each other and to the extracellular matrix by the assembly of adherens junctions (AJ) and focal adhesions (FA) respectively. We have previously shown that, in renal papillary collecting duct (CD) cells, both AJ and FA are located in sphingomyelin (SM)-enriched plasma membrane microdomains. In the present work, we investigated the involvement of SM metabolism in the preservation of the epithelial cell phenotype and tissue organization. To this end, primary cultures of renal papillary CD cells were performed...
November 8, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29125462/systematic-proteomic-analysis-of-lrrk2-mediated-rab-gtpase-phosphorylation-establishes-a-connection-to-ciliogenesis
#10
Martin Steger, Federico Diez, Herschel S Dhekne, Pawel Lis, Raja S Nirujogi, Ozge Karayel, Francesca Tonelli, Terina N Martinez, Esben Lorentzen, Suzanne R Pfeffer, Dario R Alessi, Matthias Mann
We previously reported that Parkinson's disease (PD) kinase LRRK2 phosphorylates a subset of Rab GTPases on a conserved residue in their switch-II domains (Steger, Tonelli et al., 2016) (PMID: 26824392). Here, we systematically analyzed the Rab protein family and found 14 of them (Rab3A/B/C/D, Rab5A/B/C, Rab8A/B, Rab10, Rab12, Rab29, Rab35 and Rab43) to be specifically phosphorylated by LRRK2, with evidence for endogenous phosphorylation for ten of them (Rab3A/B/C/D, Rab8A/B, Rab10, Rab12, Rab35 and Rab43)...
November 10, 2017: ELife
https://www.readbyqxmd.com/read/29125377/intraflagellar-transport-proteins-are-involved-in-thrombocyte-filopodia-formation-and-secretion
#11
Uvaraj Radhakrishnan, Abdullah Alsrhani, Hemalatha Sundaramoorthi, Gauri Khandekar, Meghana Kashyap, Jannon L Fuchs, Brian D Perkins, Yoshihiro Omori, Pudur Jagadeeswaran
Intraflagellar transport (IFT) proteins are vital for the genesis and maintenance of cilia. Our identification of ift122 transcripts in zebrafish thrombocytes that lack primary cilia was unexpected. IFT proteins serve transport in cilia, whose narrow dimensions may have necessitated the evolution of IFT from vesicular transport in ancestral eukaryotes. We hypothesized that IFTs might also facilitate transport within the filopodia that form when thrombocytes are activated. To test this possibility, we knocked down ift122 expression by injecting antisense Morpholino oligonucleotides (MOs) into zebrafish embryos...
November 10, 2017: Platelets
https://www.readbyqxmd.com/read/29122585/strong-sonic-hedgehog-signaling-in-the-mouse-ventral-spinal-cord-is-not-required-for-oligodendrocyte-precursor-cell-opc-generation-but-is-necessary-for-correct-timing-of-its-generation
#12
Hirokazu Hashimoto, Wen Jiang, Takeshi Yoshimura, Kyeong-Hye Moon, Jinwoong Bok, Kazuhiro Ikenaka
In the mouse neural tube, sonic hedgehog (Shh) secreted from the floor plate (FP) and the notochord (NC) regulates ventral patterning of the neural tube, and later is essential for the generation of oligodendrocyte precursor cells (OPCs). During early development, the NC is adjacent to the neural tube and induces ventral domains in it, including the FP. In the later stage of development, during gliogenesis in the spinal cord, the pMN domain receives strong Shh signaling input. While this is considered to be essential for the generation of OPCs, the actual role of this strong input in OPC generation remains unclear...
November 6, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/29121203/functional-characterization-of-tektin-1-in-motile-cilia-and-evidence-for-tekt1-as-a-new-candidate-gene-for-motile-ciliopathies
#13
Rebecca Ryan, Marion Failler, Madeline Louise Reilly, Meriem Garfa-Traore, Marion Delous, Emilie Filhol, Thérèse Reboul, Christine Bole-Feysot, Patrick Nitschké, Véronique Baudouin, Serge Amselem, Estelle Escudier, Marie Legendre, Alexandre Benmerah, Sophie Saunier
A child presenting with Mainzer-Saldino syndrome (MZSDS), characterized by renal, retinal and skeletal involvements, was also diagnosed with lung infections and airway ciliary dyskinesia. These manifestations suggested dysfunction of both primary and motile cilia, respectively. Targeted exome sequencing identified biallelic mutations in WDR19, encoding an IFT-A subunit previously associated with MZSDS-related chondrodysplasia, Jeune asphyxiating thoracic dysplasia and cranioectodermal dysplasia, linked to primary cilia dysfunction, and in TEKT1 which encodes tektin-1 an uncharacterized member of the tektin family, mutations of which may cause ciliary dyskinesia...
November 7, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/29118359/visualization-and-quantification-of-injury-to-the-ciliated-epithelium-using-quantitative-flow-imaging-and-speckle-variance-optical-coherence-tomography
#14
Ute A Gamm, Brendan K Huang, Emily K Mis, Mustafa K Khokha, Michael A Choma
Mucociliary flow is an important defense mechanism in the lung to remove inhaled pathogens and pollutants. Disruption of ciliary flow can lead to respiratory infections. Multiple factors, from drugs to disease can cause an alteration in ciliary flow. However, less attention has been given to injury of the ciliated epithelium. In this study, we show how optical coherence tomography (OCT) can be used to investigate injury to the ciliated epithelium in a multi-contrast setting. We used particle tracking velocimetry (PTV-OCT) to investigate the cilia-driven flow field and 3D speckle variance imaging to investigate size and extent of injury caused to the skin of Xenopus embryos...
November 8, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29118122/unique-directional-motility-of-influenza-c-virus-controlled-by-its-filamentous-morphology-and-short-range-motions
#15
Tatsuya Sakai, Hiroaki Takagi, Yasushi Muraki, Mineki Saito
Influenza virus motility is based on cooperation between two viral spike proteins, hemagglutinin (HA) and neuraminidase (NA), and is a major determinant of virus infectivity. To translocate a virus particle on the cell surface, HA molecules exchange viral receptors and NA molecules accelerate the receptor exchange of HA. This type of virus motility was recently identified in influenza A virus (IAV). To determine if other influenza virus types have a similar receptor-exchange mechanism-driven motility, we investigated influenza C virus (ICV) motility on a receptor-fixed glass surface...
November 8, 2017: Journal of Virology
https://www.readbyqxmd.com/read/29118104/conditional-deletion-of-ric-8b-in-olfactory-sensory-neurons-leads-to-olfactory-impairment
#16
Cleiton F Machado, Maíra H Nagai, Cassandra S Lyra, Thiago M Reis-Silva, André Machado Xavier, Isaias Glezer, Luciano F Felicio, Bettina Malnic
The olfactory system can discriminate a vast number of odorants. This ability derives from the existence of a large family of odorant receptors expressed in the cilia of the olfactory sensory neurons. Odorant receptors signal through the olfactory-specific G-protein subunit, Gαolf. Ric-8b, a guanine nucleotide exchange factor, interacts with Gαolf and can amplify odorant receptor signal transduction in vitro To explore the function of Ric-8b in vivo, we generated a tissue specific knockout mouse by crossing OMP-Cre transgenic mice to Ric-8b floxed mice...
November 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29116124/monocytes-from-patients-with-primary-ciliary-dyskinesia-show-enhanced-inflammatory-properties-and-produce-higher-levels-of-pro-inflammatory-cytokines
#17
M Cockx, M Gouwy, P Ruytinx, I Lodewijckx, A Van Hout, S Knoops, N Pörtner, I Ronsse, L Vanbrabant, V Godding, K De Boeck, J Van Damme, M Boon, S Struyf
Patients with Primary Ciliary Dyskinesia (PCD) suffer from recurrent upper and lower airway infections due to defects in the cilia present on the respiratory epithelium. Since chronic inflammatory conditions can cause changes in innate immune responses, we investigated whether monocytes isolated from the peripheral blood of pediatric PCD patients respond differently to inflammatory stimuli, compared to monocytes from healthy children and adults. The receptor for C5a (C5aR) was upregulated in PCD, whereas expression levels of the leukocyte chemoattractant receptors CCR1, CCR2, CCR5, BLT1 and FPR1 on PCD monocytes were similar to those on monocytes from healthy individuals...
November 7, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29115761/diagnosis-and-management-of-children-with-primary-ciliary-dyskinesia
#18
Amanda Harris
Primary ciliary dyskinesia (PCD) is a rare, genetic disorder of the motile cilia characterised by chronic lung disease, nasal and sinus disease, chronic ear infections with glue ear leading to possible hearing impairment, and subfertility. Half of patients have organ laterality defects. Despite symptoms being present from birth, PCD remains underdiagnosed. Early diagnosis and symptom management may reduce morbidity, improve quality of life and ensure children are adequately supported to meet their full potential...
September 11, 2017: Nursing Children and Young People
https://www.readbyqxmd.com/read/29113992/axonemal-dynein-assembly-requires-the-r2tp-complex-component-pontin
#19
Yuanyuan Li, Lu Zhao, Shiaulou Yuan, Jiefang Zhang, Zhaoxia Sun
Pontin (Ruvbl1) and Reptin (Ruvbl2) are closely related AAA ATPases. They are components of the Ruvbl1-Rvubl2-Tah1-Pih1 (R2TP) complexes that function as co-chaperones for the assembly of multiple macromolecular protein complexes. Here, we show that Pontin is essential for cilia motility in both zebrafish and mouse and that Pontin and Reptin function cooperatively in this process. pontin zebrafish mutants display phenotypes tightly associated with cilia defects and cilia motility is lost in a number of ciliated tissues along with a reduction in the number of both outer and inner dynein arms (ODAs and IDAs)...
November 7, 2017: Development
https://www.readbyqxmd.com/read/29112690/disrupted-epithelial-macrophage-crosstalk-via-spinster-homologue-2-mediated-s1p-signaling-may-drive-defective-macrophage-phagocytic-function-in-copd
#20
Hai B Tran, Hubertus Jersmann, Tung Thanh Truong, Rhys Hamon, Eugene Roscioli, Miranda Ween, Melissa R Pitman, Stuart M Pitson, Greg Hodge, Paul N Reynolds, Sandra Hodge
INTRODUCTION: We have previously established a link between impaired phagocytic capacity and deregulated S1P signaling in alveolar macrophages from COPD subjects. We hypothesize that this defect may include a disruption of epithelial-macrophage crosstalk via Spns2-mediated intercellular S1P signaling. METHODS: Primary alveolar macrophages and bronchial epithelial cells from COPD subjects and controls, cell lines, and a mouse model of chronic cigarette smoke exposure were studied...
2017: PloS One
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