Loïc Guillot,
Aurore Carré,
Gabor Szinnai,
Mireille Castanet,
Elodie Tron,
Francis Jaubert,
Isabelle Broutin,
François Counil,
Delphine Feldmann,
Annick Clement,
Michel Polak and
Ralph Epaud
Abstract
We identified and functionally characterized two new de novo NKX2-1 mutations c.493C>T (p.R165W) and c.786_787del2 (p.L263fs) in infants with closely similar severe interstitial lung disease (ILD), hypotonia, and congenital hypothyroidism. Functional analyses using A549 and HeLa cells revealed that...
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PMID: 20020530
PDF is available here.
Abstract
We conclude that PFC effects can be explained by modest, unspecific interactions with the plasma membrane rather than by specific interactions with intracellular targets....
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PMID: 20459693
PDF is available here.
Abstract
Pulmonary surfactant has two crucial roles in respiratory function; first, as a biophysical entity it reduces surface tension at the air water interface, facilitating gas exchange and alveolar stability during breathing, and, second, as an innate component of the lung's immune system it helps mainta...
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PMID: 20054141
PDF is available here.
Abstract
This article discusses the idea and set-up of a laboratory system for generating reproducible concentrated occupational aerosols containing metal compounds. A dust representative for 2 metal-machining workstations (an electric grinder and an electric disc cutter) was released from a fluidized-bed ge...
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PMID: 20331917
PDF is available here.
Abstract
SP-B(CTERM), a cationic, helical peptide based on the essential lung surfactant protein B (SP-B), retains a significant fraction of the function of the full-length protein. Solid-state (2)H- and (31)P-NMR were used to examine the effects of SP-B(CTERM) on mechanically oriented lipid bilayer samples....
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PMID: 19413982
PDF is available here.
Abstract
The benefits of exogenous synthetic or animal-derived surfactants for prevention or treatment of respiratory distress syndrome (RDS) are well established. Data from head-to-head trials comparing animal-derived surfactants primarily with the synthetic surfactant colfosceril suggest that the major cli...
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PMID: 19399006
PDF is available here.
Abstract
ErbB4 is a predominant heterodimer for other ErbB receptors in late fetal lung development where it participates in regulating type II cell surfactant synthesis. To further elucidate the role of ErbB4 in pulmonary alveolar epithelial cell function, the authors hypothesized that ErbB4 participates in...
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PMID: 19191103
PDF is available here.
Abstract
We report on the noninvasive identification of spectral markers of alveolar type II (ATII) cell differentiation in vitro using Raman microspectroscopy. ATII cells are progenitor cells for alveolar type I (ATI) cells in vivo, and spontaneously differentiate toward an ATI-like phenotype in culture. We...
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PMID: 18820234
PDF is available here.
Abstract
These results suggest that HIF1alpha is essential for proper lung maturation and alteration in its normal signaling during premature delivery might explain the pathophysiology of neonatal RDS....
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PMID: 18801745
PDF is available here.
Abstract
IAI was associated with an increased amniotic fluid concentration of SP-B in patients who received antenatal corticosteroids within 7 days prior to amniocentesis....
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PMID: 18828060
PDF is available here.
Abstract
We aimed to investigate if the SP may play a role in inflammatory skin diseases. Seven volunteers with psoriasis (n = 3), atopic dermatitis (n = 2), lichen planus (n = 1) and Behcet's disease (n = 1) participated in the study. Biopsies from each lesion and adjacent (approximately 2 cm distant) norma...
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PMID: 18386026
PDF is available here.
Abstract
The transition to air breathing after birth requires both anatomic and biochemical maturation of the lung. Lung morphogenesis is mediated by complex paracrine interactions between respiratory epithelial cells and mesenchymal cells that direct transcriptional programs guiding patterning and cytodiffe...
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PMID: 18599506
PDF is available here.
Abstract
A boy without symptoms up to 12 months of age started with persisting cough followed by respiratory failure at 18 months of age, resulting in mechanical ventilation because of alveolar proteinosis. Lung biopsy showed PAS-positive material. PCR was negative for CMV, Pneumocystis jiroveci and adenovir...
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PMID: 17618459
PDF is available here.
Donald McCurnin,
Steven Seidner,
Ling-Yi Chang,
Nahid Waleh,
Machiko Ikegami,
Jean Petershack,
Brad Yoder,
Luis Giavedoni,
Kurt H Albertine,
Mar Janna Dahl,
Zheng-ming Wang and
Ronald I Clyman
Abstract
Ibuprofen-induced patent ductus arteriosus closure improved pulmonary mechanics, decreased total lung water, increased epithelial sodium channel expression, and decreased the detrimental effects of preterm birth on alveolarization....
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PMID: 18450898
PDF is available here.
Abstract
The immune response to respiratory syncytial virus (RSV) infection has fascinated and frustrated investigators for decades. After adverse responses to early attempts at vaccination, it became popularly held that disease following infection was related to overly aggressive immune responses. However,...
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PMID: 17985249
PDF is available here.
Abstract
We explored the effect of intermittent hypobaric hypoxia conditions on the ability of non-immunocompromised rats infected by endotracheal route with P. carinii to clear the infection from their lungs. Control rats, inoculated or not with P. carinii, were maintained in normobaric normoxic conditions,...
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PMID: 18416247
PDF is available here.
Abstract
Comprehensive monitoring showed each treatment strategy to improve gas exchange and lung function, although the effect on PaO2 and pulmonary compliance declined over the study period in the surfactant groups. The overall improvement of the ventilation efficiency index (VEI) was significantly greater...
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PMID: 18625067
PDF is available here.
Katharine A KA Kevill,
Vineet V Bhandari,
Mika M Kettunen,
Lin L Leng,
Juan J Fan,
Yuka Y Mizue,
James D JD Dzuira,
Miguel M Reyes-Mugica,
Courtney L CL McDonald,
John A JA Baugh,
Christine L CL O'Connor,
Zubair H ZH Aghai,
Seamas C SC Donnelly,
Alia A Bazzy-Asaad and
Richard J RJ Bucala
Abstract
We demonstrate a central role for macrophage migration inhibitory factor (MIF) in lung maturation at the developmental stage when human neonates are most susceptible to RDS. We prematurely delivered mouse pups at embryonic day 18, during the early saccular stage of pulmonary development. Only 8% of...
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PMID: 18097062
PDF is available here.
Abstract
The pulmonary surfactant system constitutes an excellent example of how dynamic membrane polymorphism governs some biological functions through specific lipid-lipid, lipid-protein and protein-protein interactions assembled in highly differentiated cells. Lipid-protein surfactant complexes are assemb...
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PMID: 18515069
PDF is available here.
Abstract
Our results indicate that at 47-53 mN/m, the hydrophobic surfactant proteins destabilize the compressed monolayers and tend to limit access to the higher surface pressures at which the lipid films become metastable....
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PMID: 17720730
PDF is available here.
Abstract
The 2 doses of the Beta-PO4 + Beta-Ac mixture gave the best lung maturation. Single doses of the Beta-PO4 + Beta-Ac mixture and Beta-Ac alone also induced lung maturation. There were no consistent responses to either dose of Beta-PO4. CONCLUSION: Beta-PO4 alone is ineffective with the dosing schedul...
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PMID: 17980195
PDF is available here.
Abstract
These results suggest that VEGF might have potential therapeutic implications in enhancing fetal lung maturation....
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PMID: 17267143
PDF is available here.
Philip L Ballard,
Jeffrey D Merrill,
William E Truog,
Rodolfo I Godinez,
Marye H Godinez,
Theresa M McDevitt,
Yue Ning,
Sergio G Golombek,
Lance A Parton,
Xianqun Luan,
Avital Cnaan and
Roberta A Ballard
Abstract
We conclude that inhaled nitric oxide treatment for premature infants at risk of bronchopulmonary dysplasia does not alter surfactant recovery or protein composition and may improve surfactant function transiently....
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PMID: 17671061
PDF is available here.
Abstract
These results suggest that BLES-CHOL miscibility is better at low pi and low CHOL concentrations, while BLES-CHOLP miscibility is better at high pi and high CHOLP concentrations....
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PMID: 17434181
PDF is available here.
Abstract
Phylogenetic analyses of the families of mammalian lung surfactant proteins (SP-A, SP-B, SP-C, and SP-D) supported the hypothesis that these proteins have diverged between birds and mammals as a result of lineage-specific gene duplications and deletions. Homologs of mammalian genes encoding SP-B, SP...
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PMID: 17457583
PDF is available here.
Zhenwu Lin,
Neal J Thomas,
Marina Bibikova,
Carola Seifart,
Yunhua Wang,
Xiaoxuan Guo,
Guirong Wang,
Ekkehard Vollmer,
Torsten Goldmann,
Eliza Wickham Garcia,
Lixin Zhou,
Jian-Bing Fan and
Joanna Floros
Abstract
We speculate that SP-A1 and SP-D are subject to methylation/demethylation regulatory mechanisms and are involved in lung cancer pathogenesis by virtue of their function in innate host defense and/or regulation of inflammation....
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PMID: 17549420
PDF is available here.
Abstract
We suggest that in the early phase of paraquat injury, SP-D levels reflect alveolar damage and that de novo synthesis of SP-D takes 72 hours. Levels of SP-A, on the other hand, reflect abnormalities in the surfactant system in the late stage of paraquat intoxication. Surfactant proteins may play a r...
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PMID: 17616020
PDF is available here.
Abstract
Tissue inhibitor of metalloproteinase-3 (TIMP3) null mice develop emphysema-like airspace enlargement due to an enzymatic imbalance. This study investigates how these abnormalities alter lung mechanics and the response to 2 different mechanical ventilation strategies. Phenotypically, TIMP3 null mice...
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PMID: 17454105
PDF is available here.
Abstract
The presence of surfactant proteins (SPs), critical to local barrier and defense functions and usually associated with the lung, was revealed in adult and fetal human skin complementary deoxyribonucleic acid, in skin samples from three adult female donors and also in cultured fibroblasts, keratinocy...
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PMID: 17008883
PDF is available here.
Abstract
We hypothesized that increased basal lung expansion, caused by ventilating newborn lambs with a positive end-expiratory pressure (PEEP), would stimulate lung growth and alter alveolar epithelial cell (AEC) proportions and decrease surfactant protein mRNA levels. Two groups of lambs were sedated and...
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PMID: 17028266
PDF is available here.
Abstract
Inherited disorders of pulmonary surfactant-associated proteins are rare but provide important insights into unique mechanisms of surfactant dysfunction. Recessive loss-of-function mutations in the surfactant protein-B and the ATP-binding cassette family member A3 (ABCA3) genes present as lethal sur...
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PMID: 17575475
PDF is available here.
Abstract
In two trials of protein containing synthetic surfactants compared to animal derived surfactant extract, no statistically different clinical differences in death and chronic lung disease were noted. In general, clinical outcomes between the two groups were similar. Further well designed studies of a...
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PMID: 17943881
PDF is available here.
Abstract
In two trials of protein containing synthetic surfactants compared to animal derived surfactant extract, no statistically different clinical differences in death and chronic lung disease were noted. Further well designed studies of adequate size and power will be needed to confirm and refine these f...
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PMID: 17636826
PDF is available here.
Abstract
The metabolism, composition, structure and functions of the alveolar surfactant (AS) are described. The most adequate biophysical models for investigation of AS are considered. The principals and possibilities of three mostly used models are described in details: Monolayers, Spinning drop method and...
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PMID: 18173010
PDF is available here.
Abstract
We learn more about the molecular consequences of allelic variation, new therapies based on a new generation of surfactant diagnostics and individualized therapies may follow....
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PMID: 17127271
PDF is available here.
Abstract
We hypothesized that endogenous TGF-beta inhibits type II cell differentiation and that the stimulatory effects of glucocorticoids result in part from suppression of TGF-beta. Epithelial cells were isolated from human fetal lung and cultured under defined conditions with and without dexamethasone pl...
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PMID: 16997883
PDF is available here.
Abstract
Advances in genetic engineering have allowed the creation of animals with additional or deleted genes. New genes may be inserted in mice, specific genes inactivated or "knocked out," and more complex animals created in which genes can be turned on or off at different times in development or in diffe...
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PMID: 17142160
PDF is available here.
Abstract
We describe the approach of defining the genetic contribution to disease and discuss the polymorphisms of some genes that are associated with respiratory disease. The common allelic variants of SP-A1, SP-A2, SP-B, SP-C, and SP-D genes are associated with respiratory distress syndrome (RDS), bronchop...
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PMID: 17142161
PDF is available here.
Abstract
Since the original description of deficiency of the pulmonary surfactant in premature newborn infants by Avery and Mead in 1959, respiratory distress syndrome has most commonly been attributed to developmental immaturity of surfactant production. Studies of different ethnic groups, gender, targeted...
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PMID: 17027617
PDF is available here.
Abstract
Pulmonary surfactant proteins and lipids are required for lung function after birth. Lung immaturity and resultant surfactant deficiency cause respiratory distress syndrome, a common disorder contributing to morbidity and mortality in preterm infants. Surfactant synthesis increases prior to birth in...
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PMID: 16998587
PDF is available here.
Abstract
Although numerous studies have focused on the nature and defensive role of surfactant in the lower airways, relatively little is known about its role in the upper airways. Decreased levels of the main component of surfactant--phospholipids--have been implicated in atrophic rhinitis. The lamellar bod...
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PMID: 17040017
PDF is available here.
Abstract
We critically analyse the molecular interactions of surfactant proteins and phospholipids contributing to surfactant function. CONCLUSION: Peptide-containing surfactant provides clinical efficacy in the treatment of respiratory distress syndrome and offers promise for treating other lung diseases in...
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PMID: 16938747
PDF is available here.
Frank F Brasch,
Sven S Schimanski,
Christian C Mühlfeld,
Stefan S Barlage,
Thomas T Langmann,
Charalampos C Aslanidis,
Alfred A Boettcher,
Ashraf A Dada,
Horst H Schroten,
Eva E Mildenberger,
Eric E Prueter,
Manfred M Ballmann,
Matthias M Ochs,
Georg G Johnen,
Matthias M Griese and
Gerd G Schmitz
Abstract
ABCA3 protein expression was found to be greatly reduced or absent in 10 of 14 infants with URDS. Direct sequencing revealed distinct ABCA3 mutations clustering within vulnerable domains of the ABCA3 protein. A strong expression of precursors of surfactant protein B (pro-SP-B) but only low levels an...
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PMID: 16728712
PDF is available here.
Abstract
PURPOSE: The pharmacology, clinical efficacy, and cost considerations of exogenous lung surfactants are reviewed. SUMMARY: Exogenous pulmonary surfactants, along with advancements in ventilatory care, have significantly reduced the incidence of respiratory distress syndrome (RDS) and its related com...
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PMID: 16896079
PDF is available here.
Abstract
We demonstrate that physiologic concentrations of leptin (1 and 10 ng/mL) increase the levels of surfactant proteins (SP) A, B, and C mRNA as well as SP-A and SP-B protein in d-17 fetal rat lung explants in vitro. To determine whether leptin exerts similar effects in vivo, we administered leptin ant...
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PMID: 16864704
PDF is available here.
Abstract
Acute lung injury (ALI) is a complex syndrome involving the interplay of both environmental (such as the addition of mechanical ventilation) and genetic factors. Clinical models have identified risk factors for development and poor outcome but these strategies remain imprecise. To better understand...
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PMID: 16909372
PDF is available here.
Abstract
Multiple investigators have undertaken genetic studies in idiopathic pulmonary fibrosis populations in attempts to define genetic links to disease in hopes that this would improve understanding of disease pathogenesis and target pathways for therapy. Multiple genes have been evaluated using a candid...
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PMID: 16738199
PDF is available here.
Abstract
We quantified the effects of hormonal differentiation in vitro on the molecular specificity of cellular and secreted phospholipids from human fetal type II epithelial cells using electrospray ionization mass spectrometry. Differentiation, assessed by morphology and changes in gene expression, was ac...
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PMID: 16513897
PDF is available here.
Abstract
This study demonstrates significant alterations of all SPs in BAL fluid after allergen challenge of which SP-C was most closely related to surfactant dysfunction and the degree of the allergic inflammation....
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PMID: 16629790
PDF is available here.
Abstract
Immunogenicity assay: None of the surfactants analyzed triggered antibody immune responses against their components in any of the animals. Stability assay: The results of this study demonstrate that Butantan surfactant was as effective as Curosurf when both were submitted to the adverse circumstance...
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PMID: 16680333
PDF is available here.
Abstract
Short-term TO late in gestation induces rapid lung growth. Surfactant protein-mRNA and protein expression are not significantly altered. Thyroid transcription factor 1 and Hoxb5 are down-regulated by TO, suggesting that duration and timing of occlusion are important in balancing the effects of TO on...
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PMID: 16567192
PDF is available here.
Abstract
BALF phospholipid content was similar between adult horses and term foals, but BALF protein content was significantly decreased in term foals. Phosphatidylglycerol was significantly decreased, phosphatidylinositol was significantly increased, and the minimum surface tension was significantly increas...
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PMID: 17186857
PDF is available here.
Abstract
Gene expression of all surfactant proteins (SP-A, -B, -C and -D) was high at birth and initially declined during neonatal development, but SP-A, -B, and -D mRNA levels increased during exposure to hyperoxia compared to room-air controls. Peak levels were observed in adult lungs for SP-A, SP-C and CC...
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PMID: 16620381
PDF is available here.
Abstract
A 63-year-old man who had undergone resection of colon cancer 15 years previously was found to have a right hilar mass on chest X-ray, and an elevated serum carcinoembryonic antigen level. The hilar lymph nodes were resected with the right upper lobe, and the initial diagnosis was colon cancer metas...
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PMID: 16493539
PDF is available here.
Abstract
We performed a family based association study using a Greek study group of 71 neonates (<30 wks gestational age) from 60 families with, 52 BPD_28D and 19 BPD_36W, affected infants. Genotyping was performed using newly designed pyrosequencing assays and previously published methods. Associations betw...
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PMID: 17264398
PDF is available here.