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Surfactant deficiency

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https://www.readbyqxmd.com/read/29345196/surfactant-protein-c-dampens-inflammation-by-decreasing-jak-stat-activation-during-lung-repair
#1
Huiyan Jin, Andrzej K Ciechanowicz, Alanna R Kaplan, Lin Wang, Ping Xia Zhang, Yi-Chien Lu, Rachel E Tobin, Brooke A Tobin, Lauren Cohn, Caroline J Zeiss, Patty J Lee, Emanuela M Bruscia, Diane S Krause
Surfactant Protein C (SPC), a key component of pulmonary surfactant, also plays a role in regulating inflammation. SPC deficiency in patients and mouse models is associated with increased inflammation and delayed repair, but the key drivers of SPC-regulated inflammation in response to injury are largely unknown. This study focuses on a new mechanism of SPC as an anti-inflammatory molecule using SPC-TK/SPC-KO (surfactant protein C-thymidine kinase/surfactant protein C knockout) mice, which represent a novel sterile injury model that mimics clinical acute respiratory distress syndrome (ARDS)...
January 18, 2018: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29339408/development-of-the-swimbladder-surfactant-system-and-biogenesis-of-lysosome-related-organelles-is-regulated-by-blos1-in-zebrafish
#2
Tianbing Chen, Guili Song, Huihui Yang, Lin Mao, Zongbin Cui, Kaiyao Huang
Hermansky-Pudlak syndrome (HPS) is a human autosomal recessive disorder that is characterized by oculocutaneous albinism and a deficiency of the platelet storage pool resulting from defective biogenesis of lysosome-related organelles (LROs). To date, ten HPS genes have been identified, three of which belong to the octamer complex BLOC-1 (biogenesis of lysosome-related organelles complex 1). One subunit of the BLOC-1 complex, BLOS1, also participates in the BLOC-1-related complex (BORC). Due to lethality at the early embryo stage in BLOS1 knockout mice, the function of BLOS1 in the above two complexes and whether it has a novel function are unclear...
January 16, 2018: Genetics
https://www.readbyqxmd.com/read/29327790/iron-encapsulation-in-water-in-oil-emulsions-effect-of-ferrous-sulfate-concentration-and-fat-crystal-formation-on-oxidative-stability
#3
Nattapong Prichapan, David Julian McClements, Utai Klinkesorn
Iron deficiency is a major global human health concern. Encapsulation of iron in functional food products may help to solve this problem. However, iron is highly reactive and may promote rapid lipid oxidation in fatty foods. In this study, the effect of ferrous sulfate (0.1 to 0.5 wt%) and rice bran stearin (0 or 30 wt%) on the physical properties, oxidative stability, and encapsulation efficiency of 20 wt% water-in-oil (W/O) emulsions stabilized with polyglycerol polyricinoleate was investigated. In the presence of rice bran stearin crystals in the continuous oil phase, W/O emulsions had smaller mean droplet diameters (d ∼ 250 nm) and better physical stability than its absence (d ∼ 330 nm)...
January 12, 2018: Journal of Food Science
https://www.readbyqxmd.com/read/29320485/non-invasive-ventilation-and-surfactant-treatment-as-primary-mode-of-respiratory-support-in-surfactant-deficient-newborn-piglets
#4
Carmen Rey-Santano, Victoria E Mielgo, Miguel Angel Gomez-Solaetxe, Francesca Ricci, Federico Bianco, Fabrizio Salomone, Begoña Loureiro, Jon López de Heredia Y Goya
BACKGROUND: Nasal continuous positive airway pressure (NCPAP) and nasal intermittent positive pressure ventilation (NIPPV), forms of non-invasive ventilation (NIV) for respiratory support, are increasingly being chosen as the initial treatment for neonates with surfactant (SF) deficiency. Our objective was to compare NCPAP with NIPPV with or without SF administration as a primary mode of ventilation. METHODS: Twenty-four newborn piglets with SF-deficient lung injury produced by repetitive bronchoalveolar lavages were randomly assigned to NCPAP or NIPPV, with or without SF administration (InSurE method)...
January 10, 2018: Pediatric Research
https://www.readbyqxmd.com/read/29288965/determination-of-total-plasma-oxysterols-by-enzymatic-hydrolysis-solid-phase-extraction-and-liquid-chromatography-coupled-to-mass-spectrometry
#5
Isabel Mendiara, Celia Domeño, Cristina Nerín, Aron M Geurts, Jesús Osada, Roberto Martínez-Beamonte
The potential use of cholesterol esterases was tested to avoid alkaline hydrolysis for cleavage of plasma esterified oxysterols. The enzymatic hydrolysis was optimized by testing two sources of enzyme-Pseudomonas and bovine pancreas, presence of surfactants, incubation time and amount of enzyme. Free forms of 4β-, 7-, 24-, 25- and 27-hydroxycholesterol (HC) as well 7-ketocholesterol (7-KC) were analyzed by liquid chromatography and mass-spectrometry using the deuterated internal standard, 25-HC(d6). Enzymatic hydrolysis was more effective using the Pseudomonas enzyme and in presence of surfactants...
December 21, 2017: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/29167126/chronic-lung-injury-and-impaired-pulmonary-function-in-a-mouse-model-of-acid-ceramidase-deficiency
#6
Fabian Ps Yu, Diana Islam, Jakub Sikora, Shaalee Dworski, Jiří Gurka', Lucia Lopez-Vasquez, Mingyao Liu, Wolfgang Michael Kuebler, Thierry Levade, Haibo Zhang, Jeffrey A Medin
Farber Disease (FD) is a debilitating Lysosomal Storage Disorder (LSD) caused by a deficiency of acid ceramidase (ACDase) activity due to mutations in the gene ASAH1. Patients with ACDase deficiency may develop a spectrum of clinical phenotypes. Severe cases of FD are frequently associated with neurological involvement, failure to thrive, and respiratory complications. Mice homozygous (Asah1P361R/P361R) for an orthologous patient mutation in Asah1 recapitulate human FD. In this study, we show significant impairment in lung function including low compliance and increased airway resistance in a mouse model of ACDase deficiency...
November 22, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/29127920/uptake-and-phytotoxic-effect-of-benzalkonium-chlorides-in-lepidium-sativum-and-lactuca-sativa
#7
Adnan Hossain Khan, Mark Libby, Daniel Winnick, John Palmer, Mark Sumarah, Madhumita B Ray, Sheila M Macfie
Cationic surfactants such as benzalkonium chlorides (BACs) are used extensively as biocides in hospitals, food processing industries, and personal care products. BACs have the potential to reach the rooting zone of crop plants and BACs might thereby enter the food chain. The two most commonly used BACs, benzyl dimethyl dodecyl ammonium chloride (BDDA) and benzyl dimethyl tetradecyl ammonium chloride (BDTA), were tested in a hydroponic system to assess the uptake by and phytotoxicity to lettuce (Lactuca sativa L...
November 8, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/29067996/the-survival-of-fetal-and-bone-marrow-monocyte-derived-alveolar-macrophages-is-promoted-by-cd44-and-its-interaction-with-hyaluronan
#8
Y Dong, G F T Poon, A A Arif, S S M Lee-Sayer, M Dosanjh, P Johnson
Alveolar macrophages maintain lung homeostasis by performing important roles in immunosurveillance and lung surfactant catabolism. They express high levels of CD44 and are one of the few macrophage populations that constitutively bind hyaluronan, a ligand for CD44 and component of pericellular and extracellular matrices. Using adoptive transfer experiments and a mouse model of inflammation, we found that alveolar macrophages are initially depleted after an inflammatory insult then rapidly self-renew and return to original numbers after the resolution phase...
October 25, 2017: Mucosal Immunology
https://www.readbyqxmd.com/read/28922348/stem-cell-biology-and-regenerative-medicine-for-neonatal-lung-diseases
#9
REVIEW
Martin Kang, Bernard Thébaud
Lung diseases remain one of the main causes of morbidity and mortality in neonates. Cell therapy and regenerative medicine have the potential to revolutionize the management of life-threatening and debilitating lung diseases that currently lack effective treatments. Over the past decade, the repair capabilities of stem/progenitor cells have been harnessed to prevent/rescue lung damage in experimental neonatal lung diseases. Mesenchymal stromal cells and amnion epithelial cells exert pleiotropic effects and represent ideal therapeutic cells for bronchopulmonary dysplasia, a multifactorial disease...
October 18, 2017: Pediatric Research
https://www.readbyqxmd.com/read/28916727/inflammatory-responses-induce-an-identity-crisis-of-alveolar-macrophages-leading-to-pulmonary-alveolar-proteinosis
#10
COMPARATIVE STUDY
Risa Ebina-Shibuya, Mitsuyo Matsumoto, Makoto Kuwahara, Kyoung-Jin Jang, Manabu Sugai, Yoshiaki Ito, Ryo Funayama, Keiko Nakayama, Yuki Sato, Naoto Ishii, Yasunobu Okamura, Kengo Kinoshita, Kohei Kometani, Tomohiro Kurosaki, Akihiko Muto, Masakazu Ichinose, Masakatsu Yamashita, Kazuhiko Igarashi
Pulmonary alveolar proteinosis (PAP) is a severe respiratory disease characterized by dyspnea caused by accumulation of surfactant protein. Dysfunction of alveolar macrophages (AMs), which regulate the homeostasis of surfactant protein, leads to the development of PAP; for example, in mice lacking BTB and CNC homology 2 (Bach2). However, how Bach2 helps prevent PAP is unknown, and the cell-specific effects of Bach2 are undefined. Using mice lacking Bach2 in specific cell types, we found that the PAP phenotype of Bach2-deficient mice is due to Bach2 deficiency in more than two types of immune cells...
November 3, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28888561/delayed-presentation-and-prolonged-survival-of-a-child-with-surfactant-protein-b-deficiency
#11
Juan A López-Andreu, Antonio D Hidalgo-Santos, Miguel A Fuentes-Castelló, Nuria Mancheño-Franch, Juan A Cerón-Pérez, Maria J Esteban-Ricós, Laia Pedrola-Vidal, Lawrence M Nogee
Surfactant protein B encoding gene mutations have been related to early onset fatal respiratory distress in full-term neonates. We report a school-aged male child homozygous for a surfactant protein B encoding gene missense mutation who presented after the neonatal period. His respiratory insufficiency responded to high dose intravenous methylprednisolone and hydroxychloroquine.
September 6, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28878025/gallbladder-derived-surfactant-protein-d-regulates-gut-commensal-bacteria-for-maintaining-intestinal-homeostasis
#12
Hana Sarashina-Kida, Hideo Negishi, Junko Nishio, Wataru Suda, Yuki Nakajima, Mika Yasui-Kato, Keiko Iwaisako, Sujin Kang, Nobuyasu Endo, Hideyuki Yanai, Masataka Asagiri, Hiroshi Kida, Masahira Hattori, Atsushi Kumanogoh, Tadatsugu Taniguchi
The commensal microbiota within the gastrointestinal tract is essential in maintaining homeostasis. Indeed, dysregulation in the repertoire of microbiota can result in the development of intestinal immune-inflammatory diseases. Further, this immune regulation by gut microbiota is important systemically, impacting health and disease of organ systems beyond the local environment of the gut. What has not been explored is how distant organs might in turn shape the microbiota via microbe-targeted molecules. Here, we provide evidence that surfactant protein D (SP-D) synthesized in the gallbladder and delivered into intestinal lumen binds selectively to species of gut commensal bacteria...
September 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28873200/mac-1-deficiency-induces-respiratory-failure-by-affecting-type-i-alveolar-epithelial-cells
#13
J Wang, Y B Ci, C L Liu, H M Sun
As a β2 integrin family member, Mac-1 plays an important role in the inflammatory response. Inflammation and lung injury are closely associated, but the involvement of Mac-1 in the occurrence and development of such pathologies remains poorly understood. We aimed to investigate the relationship between Mac-1 deficiency and respiratory failure in Mac-1 knockout {Mac-1(-/-)} mice, using C57BL/6J mice as a control. The newborn survival rate of Mac-1(-/-) mice was calculated, and mouse lung tissue was treated with hematoxylin and eosin and subjected to immunofluorescent staining...
August 31, 2017: Genetics and Molecular Research: GMR
https://www.readbyqxmd.com/read/28854814/function-and-safety-of-lentivirus-mediated-gene-transfer-for-csf2ra-deficiency
#14
Miriam Hetzel, Takuji Suzuki, Anna Rafiei Hashtchin, Paritha Arumugam, Brenna Carey, Marc Schwabbauer, Alexandra Kuhn, Johann Meyer, Axel Schambach, Johannes Van Der Loo, Thomas Moritz, Bruce C Trapnell, Nico Lachmann
Hereditary pulmonary alveolar proteinosis (hPAP) is a rare disorder of pulmonary surfactant accumulation and hypoxemic respiratory failure caused by mutations in CSF2RA (encoding the granulocyte/macrophage colony-stimulating factor [GM-CSF] receptor α-chain [CD116]), which results in reduced GM-CSF-dependent pulmonary surfactant clearance by alveolar macrophages. While no pharmacologic therapy currently exists for hPAP, it was recently demonstrated that endotracheal instillation of wild-type or gene-corrected mononuclear phagocytes (pulmonary macrophage transplantation [PMT]) results in a significant and durable therapeutic efficacy in a validated murine model of hPAP...
August 30, 2017: Human Gene Therapy Methods
https://www.readbyqxmd.com/read/28839101/hyperoxia-treatment-of-trek-1-trek-2-traak-deficient-mice-is-associated-with-a-reduction-in-surfactant-proteins
#15
Andreas Schwingshackl, Benjamin Lopez, Bin Teng, Charlean L Luellen, Florian Lesage, John Belperio, Riccardo Olcese, Christopher M Waters
We previously proposed a role for the 2-pore domain potassium (K2P) channel TREK-1 in hyperoxia (HO)-induced lung injury. To determine whether redundancy between the 3 TREK isoforms (TREK-1, TREK-2, TRAAK) could protect from HO-induced injury, we now examined the effect of deletion of all 3 TREK isoforms in a clinically relevant scenario of prolonged HO exposure and mechanical ventilation (MV). We exposed WT and TREK-1/TREK-2/TRAAK-deficient (triple ko) mice to either room air, 72 hours HO, MV (high and low tidal volume), or a combination of HO+MV, and measured quasi-static lung compliance, BAL protein concentration, histologic lung injury scores (LIS), cellular apoptosis, and cytokine levels...
August 24, 2017: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/28837561/histologic-and-biochemical-alterations-predict-pulmonary-mechanical-dysfunction-in-aging-mice-with-chronic-lung-inflammation
#16
Christopher B Massa, Angela M Groves, Smita U Jaggernauth, Debra L Laskin, Andrew J Gow
Both aging and chronic inflammation produce complex structural and biochemical alterations to the lung known to impact work of breathing. Mice deficient in surfactant protein D (Sftpd) develop progressive age-related lung pathology characterized by tissue destruction/remodeling, accumulation of foamy macrophages and alteration in surfactant composition. This study proposes to relate changes in tissue structure seen in normal aging and in chronic inflammation to altered lung mechanics using a computational model...
August 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/28725012/phycocyanin-attenuates-pulmonary-fibrosis-via-the-tlr2-myd88-nf-%C3%AE%C2%BAb-signaling-pathway
#17
Chengcheng Li, Yan Yu, Wenjun Li, Bo Liu, Xudong Jiao, Xinyu Song, Changjun Lv, Song Qin
Our aim was to investigate the effects of phycocyanin (PC) on bleomycin (BLM)-induced pulmonary fibrosis (PF). In this study, C57 BL/6 wild-type (WT) mice and toll-like receptor (TLR) 2 deficient mice were treated with PC for 28 days following BLM exposure. Serum and lung tissues were collected on days 3, 7 and 28. Data shows PC significantly decreased the levels of hydroxyproline (HYP), vimentin, surfactant-associated protein C (SP-C), fibroblast specific protein-1 (S100A4) and α-smooth muscle actin (α-SMA) but dramatically increased E-cadherin and podoplanin (PDPN) expression on day 28...
July 19, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28647590/differences-in-the-coronal-proteome-acquired-by-particles-depositing-in-the-lungs-of-asthmatic-versus-healthy-humans
#18
Abhinav Kumar, Elif Melis Bicer, Paul Pfeffer, Marco P Monopoli, Kenneth A Dawson, Jonny Eriksson, Katarina Edwards, Steven Lynham, Matthew Arno, Annelie F Behndig, Anders Blomberg, Graham Somers, Dave Hassall, Lea Ann Dailey, Ben Forbes, Ian Mudway
Most inhaled nanomedicines in development are for the treatment of lung disease, yet little is known about their interaction with the respiratory tract lining fluids (RTLFs). Here we combined the use of nano-silica, as a protein concentrator, with label-free snapshot proteomics (LC-MS/MS; key findings confirmed by ELISA) to generate a quantitative profile of the RTLF proteome and provided insight into the evolved corona; information that may be used in future to improve drug targeting to the lungs by inhaled medicines...
June 22, 2017: Nanomedicine: Nanotechnology, Biology, and Medicine
https://www.readbyqxmd.com/read/28592390/greywater-characterization-and-loadings-physicochemical-treatment-to-promote-onsite-reuse
#19
C Noutsopoulos, A Andreadakis, N Kouris, D Charchousi, P Mendrinou, A Galani, I Mantziaras, E Koumaki
Greywater is the wastewater produced in bathtubs, showers, hand basins, kitchen sinks, dishwashers and laundry machines. Segregation of greywater and blackwater and on site greywater treatment in order to promote its reuse for toilet flushing and/or garden irrigation is an interesting option especially in water deficient areas. The objective of this study was to characterize the different greywater sources in Greek households and to evaluate the performance of alternative physicochemical treatment systems to treat several types of greywater...
June 4, 2017: Journal of Environmental Management
https://www.readbyqxmd.com/read/28581337/featured-article-electroporation-mediated-gene-delivery-of-surfactant-protein-b-sp-b-restores-expression-and-improves-survival-in-mouse-model-of-sp-b-deficiency
#20
Rebecca C Barnett, Xin Lin, Michael Barravecchia, Rosemary A Norman, Karen L de Mesy Bentley, Fabeha Fazal, Jennifer L Young, David A Dean
Surfactant Protein B Deficiency is a rare but lethal monogenetic, congenital lung disease of the neonate that is unresponsive to any treatment except lung transplantation. Based on the potential that gene therapy offers to treat such intractable diseases, our objective was to test whether an electroporation-based gene delivery approach could restore surfactant protein B expression and improve survival in a compound knockout mouse model of surfactant protein B deficiency. Surfactant protein B expression can be shut off in these mice upon withdrawl of doxycycline, resulting in decreased levels of surfactant protein B within four days and death due to lung dysfunction within four to seven days...
July 2017: Experimental Biology and Medicine
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