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Surfactant deficiency

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https://www.readbyqxmd.com/read/28207927/attenuated-airway-hyperresponsiveness-and-mucus-secretion-in-hdm-exposed-igf1r-deficient-mice
#1
S Piñeiro-Hermida, J A Gregory, I P López, R Torrens, C Ruíz-Martínez, M Adner, J G Pichel
BACKGROUND: Asthma is a common chronic lung disease characterized by airflow obstruction, airway hyperresponsiveness (AHR) and inflammation. IGFs have been reported to play a role in asthma but little is known about how the Insulin-like Growth Factor 1 Receptor (IGF1R) affects asthma pathobiology. METHODS: Female Igf1r-deficient and control mice were intranasally challenged with house dust mite (HDM) extract or PBS five days per week for four weeks. Lung function measurements, and bronchioalveolar lavage fluid (BALF), serum and lungs were collected on day 28 for further cellular, histological and molecular analysis...
February 16, 2017: Allergy
https://www.readbyqxmd.com/read/28188034/follistatin-like-1-expression-is-decreased-in-the-alveolar-epithelium-of-hypoplastic-rat-lungs-with-nitrofen-induced-congenital-diaphragmatic-hernia
#2
Toshiaki Takahashi, Julia Zimmer, Florian Friedmacher, Prem Puri
BACKGROUND/PURPOSE: Pulmonary hypoplasia (PH), characterized by incomplete alveolar development, remains a major therapeutic challenge associated with congenital diaphragmatic hernia (CDH). Follistatin-like 1 (Fstl1) is a crucial regulator of alveolar formation and maturation, which is strongly expressed in distal airway epithelium. Fstl1-deficient mice exhibit reduced airspaces, impaired alveolar epithelial cell differentiation, and insufficient production of surfactant proteins similar to PH in human CDH...
January 28, 2017: Journal of Pediatric Surgery
https://www.readbyqxmd.com/read/28185902/lung-remodeling-in-aging-surfactant-protein-d-deficient-mice
#3
Jan Philipp Schneider, Martina Arkenau, Lars Knudsen, Dirk Wedekind, Matthias Ochs
Pulmonary surfactant, a mixture of lipids and proteins at the air-liquid interface of alveoli, prevents the lungs from collapsing due to surface tension. One constituent is surfactant-associated protein-D (SP-D), a protein involved in surfactant homeostasis and innate immunity. Mice deficient in SP-D (SP-D (-/-)) have been described as developing a characteristic phenotype which affects the surfactant system (including changes in the intra-cellular and intra-alveolar surfactant pool, alveolar epithelial type II cells and alveolar macrophages), lung architecture and its inflammatory state (development of an emphysema-like pathology, inflammatory cell infiltration)...
February 6, 2017: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28152192/effects-of-tracheal-occlusion-with-retinoic-acid-administration-on-normal-lung-development
#4
Delabaere Amélie, Marceau Geoffroy, Coste Karen, Blanchon Loïc, Déchelotte Pierre Jean, Blanc Pierre, Sapin Vincent, Gallot Denis
INTRODUCTION: Tracheal occlusion (TO) is an investigational therapy for severe congenital diaphragmatic hernia (CDH) that decreases pulmonary hypoplasia, but sustained TO also induces deficient surfactant synthesis. Intramuscular maternal administration of retinoic acid (RA) in a surgical rabbit model of CDH showed a beneficial effect on lung maturation. We evaluated the potential of RA delivery into the trachea and studied the combined effects of TO and RA on normal lung development...
February 2, 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28049128/immune-reconstitution-inflammatory-syndrome-associated-with-pulmonary-pathogens
#5
REVIEW
Radha Gopal, Rekha R Rapaka, Jay K Kolls
Immune reconstitution inflammatory syndrome (IRIS) is an exaggerated immune response to a variety of pathogens in response to antiretroviral therapy-mediated recovery of the immune system in HIV-infected patients. Although IRIS can occur in many organs, pulmonary IRIS, associated with opportunistic infections such as Mycobacterium tuberculosis and Pneumocystis jirovecii, is particularly associated with high morbidity and mortality. The pathology of IRIS is associated with a variety of innate and adaptive immune factors, including CD4(+) T-cells, CD8(+) T-cells, γδ T-cells, natural killer cells, macrophages, the complement system and surfactant proteins, Toll-like receptors and pro-inflammatory cytokines and chemokines...
January 2017: European Respiratory Review: An Official Journal of the European Respiratory Society
https://www.readbyqxmd.com/read/28034695/aberrant-lung-remodeling-in-a-mouse-model-of-surfactant-dysregulation-induced-by-modulation-of-the-abca3-gene
#6
Michael F Beers, Lars Knudsen, Yaniv Tomer, Julian Maronn, Ming Zhao, Matthias Ochs, Surafel Mulugeta
The lipid transporter, ATP binding cassette class A3 (ABCA3), plays a critical role in the biogenesis of alveolar type 2 (AT2) cell lamellar bodies (LBs). A relatively large number of mutations in the ABCA3 gene have been identified in association with diffuse parenchymal lung disease (DPLD), the most common of which is a missense mutation (valine substitution for lysine at residue 292 (ABCA3(E292V))) that leads to functional impairment of the transporter in vitro. The consequences of ABCA3(E292)(V) gene expression in vivo are unknown...
December 26, 2016: Annals of Anatomy, Anatomischer Anzeiger: Official Organ of the Anatomische Gesellschaft
https://www.readbyqxmd.com/read/28031836/pulmonary-alveolar-proteinosis-a-case-report-and-world-literature-review
#7
Armando J Huaringa, Wassem H Francis
Pulmonary alveolar proteinosis (PAP) is a lung disorder which was first described in 1958 by Rosen et al. and is indeed rare disease with a prevalence of 0.1 per 100,000 individuals. PAP is characterized by abnormal accumulation of pulmonary surfactant in the alveolar space, which impairs gas exchange leading to a severe hypoxemia. Pulmonary surfactant is an insoluble proteinaceous material that is rich in lipids and stains positive with periodic acid-Schiff (PAS). The most common type of PAP is the so-called autoimmune or idiopathic type...
November 2016: Respirology Case Reports
https://www.readbyqxmd.com/read/28028230/ohr-plays-a-central-role-in-bacterial-responses-against-fatty-acid-hydroperoxides-and-peroxynitrite
#8
Thiago G P Alegria, Diogo A Meireles, José R R Cussiol, Martín Hugo, Madia Trujillo, Marcos Antonio de Oliveira, Sayuri Miyamoto, Raphael F Queiroz, Napoleão Fonseca Valadares, Richard C Garratt, Rafael Radi, Paolo Di Mascio, Ohara Augusto, Luis E S Netto
Organic hydroperoxide resistance (Ohr) enzymes are unique Cys-based, lipoyl-dependent peroxidases. Here, we investigated the involvement of Ohr in bacterial responses toward distinct hydroperoxides. In silico results indicated that fatty acid (but not cholesterol) hydroperoxides docked well into the active site of Ohr from Xylella fastidiosa and were efficiently reduced by the recombinant enzyme as assessed by a lipoamide-lipoamide dehydrogenase-coupled assay. Indeed, the rate constants between Ohr and several fatty acid hydroperoxides were in the 10(7)-10(8) M(-1)⋅s(-1) range as determined by a competition assay developed here...
January 10, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28025703/the-biology-of-the-abca3-lipid-transporter-in-lung-health-and-disease
#9
REVIEW
Michael F Beers, Surafel Mulugeta
The lipid transporter, ATP-binding cassette class A3 (ABCA3), is a highly conserved multi-membrane-spanning protein that plays a critical role in the regulation of pulmonary surfactant homeostasis. Mutations in ABCA3 have been increasingly recognized as one of the causes of inherited pulmonary diseases. These monogenic disorders produce familial lung abnormalities with pathological presentations ranging from neonatal surfactant-deficiency-induced respiratory failure to childhood or adult diffuse parenchymal lung diseases for which specific treatment modalities remain limited...
December 26, 2016: Cell and Tissue Research
https://www.readbyqxmd.com/read/27973472/in-vitro-and-in-vivo-comparison-between-poractant-alfa-and-the-new-generation-synthetic-surfactant-chf5633
#10
Francesca Ricci, Xabier Murgia, Roberta Razzetti, Nicola Pelizzi, Fabrizio Salomone
BACKGROUND: CHF5633 is a new generation synthetic surfactant containing both SP-B and SP-C analogues developed for the treatment of respiratory distress syndrome. Here, the optimal dose and its performance in comparison to the animal-derived surfactant poractant alfa were investigated. METHODS: In vitro surfactant activity was determined by means of the Wilhelmy balance and the capillary surfactometer. The dose-finding study was performed in preterm rabbits with severe surfactant deficiency...
December 14, 2016: Pediatric Research
https://www.readbyqxmd.com/read/27916681/surfactant-protein-a-deficiency-exacerbates-renal-interstitial-fibrosis-following-obstructive-injury-in-mice
#11
Shaojiang Tian, Chenxiao Li, Ran Ran, Shi-You Chen
Renal interstitial fibrosis is an inevitable consequence of virtually every type of chronic kidney disease. The underlying mechanisms, however, are not completely understood. In the present study, we identified surfactant protein A (SP-A) as a novel protein factor involved in the renal fibrosis induced by unilateral ureter obstruction (UUO). UUO induced SP-A expression in mouse kidney epithelium, likely due to the increased acidic stress and inflammation. Interestingly, SP-A deficiency aggravated UUO-prompted kidney structural damage, macrophage accumulation, and tubulointerstitial fibrosis...
February 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/27866256/surfactant-free-microemulsion-electrokinetic-chromatography-sf-meekc-with-uv-and-ms-detection-a-novel-approach-for-the-separation-and-esi-ms-detection-of-neutral-compounds
#12
Urška Mohorič, Andrea Beutner, Sebastian Krickl, Didier Touraud, Werner Kunz, Frank-Michael Matysik
Microemulsion electrokinetic chromatography (MEEKC) is a powerful tool to separate neutral species based on differences in their hydrophobic and hydrophilic properties. However, as a major drawback the conventionally used SDS based microemulsions are not compatible with electrospray ionization mass spectrometry (ESI-MS). In this work, a surfactant-free microemulsion (SFME) consisting of water, ethanol, and 1-octanol is used for surfactant-free microemulsion electrokinetic chromatography (SF-MEEKC). Ammonium acetate was added to the SFME enabling electrophoretic separations...
December 2016: Analytical and Bioanalytical Chemistry
https://www.readbyqxmd.com/read/27836807/surfactant-protein-a-a-key-player-in-lung-homeostasis
#13
Nadia Nathan, Jessica Taytard, Philippe Duquesnoy, Guillaume Thouvenin, Harriet Corvol, Serge Amselem, Annick Clement
The respiratory tract is continually exposed to various insults that are a permanent threat to the maintenance of lung homeostasis. Repair of the parenchyma structure, particularly of the alveolar epithelium, requires complex cellular strategies. Among the molecular components that play an important role in these processes are the surfactant proteins (SPs), particularly SP-A. The present review examines current evidence regarding the role of SP-A in lung host defence mechanisms through its implication in innate/adaptive immunity of the lung and epithelium integrity and repair...
December 2016: International Journal of Biochemistry & Cell Biology
https://www.readbyqxmd.com/read/27830194/effect-of-high-fat-diet-on-pulmonary-expression-of-parathyroid-hormone-related-protein-and-its-downstream-targets
#14
Learta Oruqaj, Svenja Forst, Rolf Schreckenberg, Javier Inserte, Marcos Poncelas, Jordi Bañeras, David Garcia-Dorado, Susanne Rohrbach, Klaus-Dieter Schlüter
AIMS: Parathyroid hormone-related protein (PTHrP) is involved in lung development and surfactant production. The latter one requires a paracrine interaction between type II alveolar cells and lipofibroblasts in which leptin triggers PTHrP-induced effects. Whether increased plasma leptin levels, as they occur in high fat diet, modify the expression of PTHrP remains unclear. Furthermore, the effect of high fat diet under conditions of forced pulmonary remodelling such as response to post myocardial infarction remains to be defined...
October 2016: Heliyon
https://www.readbyqxmd.com/read/27812430/synthetic-lung-surfactants-containing-sp-b-and-sp-c-peptides-plus-novel-phospholipase-resistant-lipids-or-glycerophospholipids
#15
Robert H Notter, Rohun Gupta, Adrian L Schwan, Zhengdong Wang, Mohanad Gh Shkoor, Frans J Walther
BACKGROUND: This study examines the biophysical and preclinical pulmonary activity of synthetic lung surfactants containing novel phospholipase-resistant phosphonolipids or synthetic glycerophospholipids combined with Super Mini-B (S-MB) DATK and/or SP-Css ion-lock 1 peptides that replicate the functional biophysics of surfactant proteins (SP)-B and SP-C. Phospholipase-resistant phosphonolipids used in synthetic surfactants are DEPN-8 and PG-1, molecular analogs of dipalmitoyl phosphatidylcholine (DPPC) and palmitoyl-oleoyl phosphatidylglycerol (POPG), while glycerophospholipids used are active lipid components of native surfactant (DPPC:POPC:POPG 5:3:2 by weight)...
2016: PeerJ
https://www.readbyqxmd.com/read/27797625/retraction-nitric-oxide-mediates-relative-airway-hyporesponsiveness-to-lipopolysaccharide-in-surfactant-protein-a-deficient-mice
#16
(no author information available yet)
No abstract text is available yet for this article.
2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27793798/epithelial-disruption-of-gab1-perturbs-surfactant-homeostasis-and-predisposes-mice-to-lung-injuries
#17
Kai Wang, Shenlu Qin, Zuyu Liang, Yun Zhang, Yingchun Xu, An Chen, Xiaohong Guo, Hongqiang Cheng, Xue Zhang, Yuehai Ke
GRB2-associated-binding protein 1 (Gab1) belongs to Gab adaptor family, which integrates multiple signals in response to the epithelial growth factors. Recent genetic studies identified genetic variants of human Gab1 gene as potential risk factors of asthmatic inflammation. However, the functions of Gab1 in lungs remain largely unknown. Alveolar type-II cells (AT-IIs) are responsible for surfactant homeostasis and essentially regulate lung inflammation following various injuries (3). In this study, in vitro knockdown of Gab1 was shown to decrease the surfactant proteins (SPs) levels in AT-IIs...
December 1, 2016: American Journal of Physiology. Lung Cellular and Molecular Physiology
https://www.readbyqxmd.com/read/27783217/lung-surfactant-metabolism-early-in-life-early-in-disease-and-target-in-cell-therapy
#18
REVIEW
Elena Lopez-Rodriguez, Gemma Gay-Jordi, Adele Mucci, Nico Lachmann, Anna Serrano-Mollar
Lung surfactant is a complex mixture of lipids and proteins lining the alveolar epithelium. At the air-liquid interface, surfactant lowers surface tension, avoiding alveolar collapse and reducing the work of breathing. The essential role of lung surfactant in breathing and therefore in life, is highlighted by surfactant deficiency in premature neonates, which causes neonatal respiratory distress syndrome and results in early death after birth. In addition, defects in surfactant metabolism alter lung homeostasis and lead to disease...
October 25, 2016: Cell and Tissue Research
https://www.readbyqxmd.com/read/27780343/gene-editing-and-genetic-lung-disease-basic-research-meets-therapeutic-application
#19
Deepthi Alapati, Edward E Morrisey
While our understanding of the genetics and pathology of congenital lung diseases such as surfactant protein deficiency, cystic fibrosis and alpha 1 antitrypsin deficiency is extensive, treatment options are lacking. Since the lung is a barrier organ in direct communication with the external environment, targeted delivery of gene corrective technologies to the respiratory system via intra-tracheal or intranasal routes is an attractive option for therapy. CRISPR/Cas9 gene editing technology is a promising approach to repair or inactivate disease causing mutations...
October 25, 2016: American Journal of Respiratory Cell and Molecular Biology
https://www.readbyqxmd.com/read/27775123/narrow-band-red-emitting-phosphor-batif6-mn-4-preparation-characterization-and-application-for-warm-white-led-devices
#20
Xiaoli Gao, Yan Song, Guixia Liu, Xiangting Dong, Jinxian Wang, Wensheng Yu
As a new class of non-rare-earth red phosphors for high-efficiency warm white light-emitting diodes (white LEDs), Mn(4+) ion activated fluoride compounds have been extensively investigated recently and hold the potential to supersede commercial rare earth doped nitride phosphors. Herein, a series of Mn(4+) ions doped BaTiF6 phosphors have been prepared via the hydrothermal route using citric acid as a surfactant. After a systematic investigation, we illustrate the effects of reaction time, nominal concentration of HF solution, and reaction temperature on the luminescence performance of the phosphor...
October 24, 2016: Dalton Transactions: An International Journal of Inorganic Chemistry
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