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Pulmonary Surfactant-Associated Protein C (0):
Recent article
Pulmonary Surfactant-Associated Protein C
Pediatric Research (68)3 2010
Abstract
We used complete resequencing of SFTPC and its promoter, genotyping, and logistic regression to identify 80 single nucleotide polymorphisms (SNPs). Three promoter SNPs were statistically associated with neonatal RDS among European descent infants. To assess the transcriptional effects of these three...
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PMID: 20539253
PDF is available here.
PDF is available here.
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Critical Care Medicine (38)7 2010
Abstract
Critical Care Medicine (38)7 2010
Abstract
The described dry powder aerosolizer may be applicable to surfactant therapy of acute lung injury/acute respiratory distress syndrome. This conclusion is based on four main factors. High doses comparable to those used for intratracheal instillation in humans can be generated within a relatively shor...
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PMID: 20400897
PDF is available here.
PDF is available here.
Abstract
Amyloid consists of cross-beta-sheet fibrils and is associated with about 25 human diseases, including several neurodegenerative diseases, systemic and localized amyloidoses and type II diabetes mellitus. Amyloid-forming proteins differ in structures and sequences, and it is to a large extent unknow...
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PMID: 20494101
PDF is available here.
PDF is available here.
Abstract
We have previously differentiated lung epithelium from human and murine embryonic stem cells (mESCs) and are now exploring the potential applications of these cells, including in the engineering of lung tissue constructs. In this study, we hypothesized that the differentiation and maintenance of lun...
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PMID: 20001250
PDF is available here.
PDF is available here.
Pediatric Research (67)5 2010
Abstract
We assessed the effects of iNO and hyperoxia, independently and concurrently, on NO-cGMP signaling pathway and differentiation. Exposure to iNO increased cGMP levels by 40-fold after 3 d and by 8-fold after 5 d despite constant expression of phosphodiesterase-5 (PDE5). The levels of cGMP declined si...
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PMID: 20098340
PDF is available here.
PDF is available here.
Biophysical Journal (98)8 2010
Abstract
We used x-ray diffraction to investigate how the physiological mixture of these proteins affects structures formed by 1-palmitoyl-2-oleoyl phosphatidylethanolamine, which by itself undergoes the lamellar-to-inverse hexagonal phase transition at 71 degrees C. In amounts as low as 0.03% (w:w) and at t...
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PMID: 20409474
PDF is available here.
PDF is available here.
Pneumonologia i alergologia polska : organ Polskiego Towarzystwa Ftyzjopneumonologicznego, Polskiego Towarzystwa Alergologicznego, i Instytutu Gruzlicy i Chorob Pluc (78)3 2010
Abstract
The interstitial lung disease in children is a group of disorders heterogeneous in its etiology and pathogenesis. In differential diagnosis infectious, immunological and metabolic factors must be taken into consideration, as well as a genetically determined surfactant protein C deficiency. In the pa...
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PMID: 20461694
PDF is available here.
PDF is available here.
Pneumonologia i alergologia polska : organ Polskiego Towarzystwa Ftyzjopneumonologicznego, Polskiego Towarzystwa Alergologicznego, i Instytutu Gruzlicy i Chorob Pluc (78)3 2010
Abstract
Etiology and pathogenesis of the interstitial lung disease in children result from a heterogeneous group of infectious, immunological and metabolic factors. In children an important role plays a surfactant protein B and C deficiency. SP-C deficiency is determined by it's defective synthesis or impai...
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PMID: 20461691
PDF is available here.
PDF is available here.
Zhonghua Yixue Zazhi (89)46 2009
Abstract
PEEP ventilation can improve the oxygenation indices and lung compliance in SW-RDS animals. And such an effect is correlated with both the mRNA expressions of SP-B/SP-C and mechanical distension....
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PMID: 20193365
PDF is available here.
PDF is available here.
Abstract
To detect the cancer stem cells and to evaluate their prognostic implication in patients with lung adenocarcinoma.
Three phenotypic markers of cancer stem cells (SP-C, CCSP and OCT4) in lung adenocarcinoma were detected by immunofluorecence staining. The correlation among the clinicopathological par...
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PMID: 20137348
PDF is available here.
PDF is available here.
Abstract
We have previously developed a transgenic mouse model in which expression of a soluble dominant-negative FGF receptor (dnFGFR) in the prenatal period results in reduced alveolar septae formation and subsequent alveolar simplification. Retinoic acid (RA), a biologically active derivative of vitamin A...
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PMID: 19502291
PDF is available here.
PDF is available here.
Abstract
Our results confirm that SFTPC mutations are a frequent cause of diffuse lung disease, and that I73T is the most frequent SFTPC mutation associated with diffuse lung disease....
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PMID: 19443464
PDF is available here.
PDF is available here.
Abstract
Patients with mutations in the pulmonary surfactant protein C (SP-C) gene develop interstitial lung disease and pulmonary exacerbations associated with viral infections including respiratory syncytial virus (RSV). Pulmonary infection with RSV caused more severe interstitial thickening, air space con...
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PMID: 19304906
PDF is available here.
PDF is available here.
Abstract
We exposed newborn wild-type and NADPH oxidase p47(phox) subunit null (p47(phox-/-)) mice to air or acute hyperoxia (95% O(2)) for up to 11 days. Hyperoxia-induced pulmonary neutrophil influx was similar in wild-type and p47(-/-) mice at postnatal days (P) 7 and 11. Macrophages were decreased in wil...
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PMID: 19411313
PDF is available here.
PDF is available here.
Journal of Immunology (182)3 2009
Abstract
We show that, in fact, TSLP alone only causes a weak innate response that is insufficient for development of full airway inflammatory disease. Complete disease development requires both TSLP and antigenic stimulation. These data suggest that the spontaneous lung inflammation observed in SPC-TSLP mic...
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PMID: 19155513
PDF is available here.
PDF is available here.
Abstract
Surfactant deficiency and the resultant respiratory distress syndrome (RDS) seen in preterm infants is a major cause of respiratory morbidity in this population. Until recently, the contribution of surfactant to respiratory morbidity in infancy was limited to the neonatal period. It is now recognise...
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PMID: 19252722
PDF is available here.
PDF is available here.
Developmental Biology (325)1 2009
Abstract
We report that DOX-fed SP-C-rtTA mice during the period in which Type II cells differentiate results in cellular toxicity that may have confounded the interpretation of previous reports using this line. These effects included impaired alveologenesis, loss/reduction in expression of surfactant-associ...
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PMID: 19013447
PDF is available here.
PDF is available here.
Respiratory Research (10) 2009
Abstract
Expression of CC16 and NKX2.1 showed a 1,000- and 10,000- fold increase at day 10 of differentiation. Other lung markers such as SP-C and Aquaporin 5 had the highest expression after twenty days of culture, as well as two markers for ciliated cells, FOXJ1 and beta-tubulin IV. The results from qRT-PC...
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PMID: 19891764
PDF is available here.
PDF is available here.
Abstract
In the model group HoxB5 expression significantly decreased 7, 14 and 21 days after hyperoxia exposure. SPC expression decreased 3 days after hyperoxia exposure but increased significantly 7, 14 and 21 days after hyperoxia exposure as compared to the control group. AQP5 expression was progressively...
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PMID: 19149924
PDF is available here.
PDF is available here.
Abstract
Mutations in the genes encoding the surfactant proteins B and C (SP-B and SP-C) and the phospholipid transporter, ABCA3, are associated with respiratory distress and interstitial lung disease in the pediatric population. Expression of these proteins is regulated developmentally, increasing with gest...
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PMID: 19220077
PDF is available here.
PDF is available here.
Abstract
We tested the hypothesis that increased GR expression targeted to type II alveolar epithelial cells would improve outcome in hyperoxia-induced lung injury. Human GR with a mitochondrial targeting sequence was targeted to mouse type II cells using the SPC promoter. Two transgenic lines were identifie...
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PMID: 18566333
PDF is available here.
PDF is available here.
Abstract
We hypothesized that SUMOylation down-regulated PLAGL2-activated SP-C promoter activity. Unexpectedly, the SUMO-conjugating enzyme Ubc9 enhanced, rather than reduced, PLAGL2 activated promoter activity but did not affect TTF-1 activation of the promoter. Ubc9 mutant (Ubc9-C93S) defective in SUMO-con...
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PMID: 18655774
PDF is available here.
PDF is available here.
Biophysical Journal (95)5 2008
Abstract
We examined surfactant membrane models containing palmitoylated and nonpalmitoylated synthetic peptides, based on the N-terminal SP-C sequence, in dipalmitoylphosphatidylcholine (DPPC)/egg phosphatidylglycerol (7:3, w/w) by (2)H-NMR. Perturbations of lipid properties by the peptide versions were com...
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PMID: 18502795
PDF is available here.
PDF is available here.
Developmental Biology (320)1 2008
Abstract
We generated a transgenic mouse model in which a doxycycline inducible HA-tagged mouse Elf5 transgene was placed under the control of the lung epithelium-specific human SFTPC promoter. We found that expressing high levels of ELF5 during early lung development disrupted branching morphogenesis and pr...
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PMID: 18544451
PDF is available here.
PDF is available here.
Abstract
We report a 15-year-old boy with the UIP pattern of pulmonary fibrosis who had mutations in the adenosine triphosphate-binding-cassette-A3 gene. Discovery of how genetic mutations of proteins involved in surfactant biosynthesis lead to progressive fibrosis will have implications for the understandin...
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PMID: 18628224
PDF is available here.
PDF is available here.
Journal of Immunology (181)1 2008
Abstract
To determine the role of surfactant protein C (SP-C) in host defense, SP-C-deficient (Sftpc-/-) mice were infected with the pulmonary pathogen Pseudomonas aeruginosa by intratracheal injection. Survival of young, postnatal day 14 Sftpc-/- mice was decreased in comparison to Sftpc+/+ mice. The sensit...
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PMID: 18566429
PDF is available here.
PDF is available here.
Abstract
We wanted to investigate the effects in a surgical model, where the retinoid signaling pathway is not primarily disrupted by the toxic agent. We created DH in fetal rabbits at day 23 of gestation, administrated to the does all trans-retinoic acid (ATRA) or vehicle (VHC) intramuscularly for 8 consecu...
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PMID: 18435480
PDF is available here.
PDF is available here.
Pediatric Research (63)6 2008
Abstract
We determined the frequencies of these mutations in ethnically diverse population and disease-based cohorts using restriction enzyme analysis (121ins2 and E292V) and a 5' nuclease assay (I73T) in DNA samples from population-based cohorts in Missouri, Norway, South Korea, and South Africa, and from a...
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PMID: 18317237
PDF is available here.
PDF is available here.
Abstract
We investigated a potential mechanism of AEC dysfunction suggested to result from mutant SFTPC expression: induction of endoplasmic reticulum (ER) stress and the unfolded protein response (UPR). We evaluated biopsies from 23 IPF patients (including 3 family members with L188Q SFTPC mutations, 10 ind...
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PMID: 18390830
PDF is available here.
PDF is available here.
Abstract
We explored the role of interactions between Foxp2 and the homeodomain TF Nkx2.1 that may contribute to the marked reduction in SP-C expression accompanying phenotypic transition of alveolar epithelial type II (AT2) to type I (AT1) cells. Foxp2 dose-dependently inhibited Nkx2.1-mediated activation o...
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PMID: 18239190
PDF is available here.
PDF is available here.
Biochimica et Biophysica Acta (1778)5 2008
Abstract
We propose that palmitoylation may be important to promote and facilitate association of SP-C and SP-C-containing membranes with ordered lipid structures such as those potentially existing in highly compressed states of the interfacial surfactant film....
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PMID: 18339301
PDF is available here.
PDF is available here.
Abstract
Abstract
We synthesized, purified, and performed in vitro testing of two classes of peptoid SP-C mimics: those having a rigid alpha-chiral aromatic helix and those having a biomimetic alpha-chiral aliphatic helix. The length of the two classes of mimics was also systematically altered. Circular dichroism spe...
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PMID: 18197709
PDF is available here.
PDF is available here.
Characterization of bovine surfactant proteins B and C by electrospray ionization mass spectrometry.
Abstract
Bovine surfactant proteins B (SP-B) and C (SP-C) were analyzed by nano-electrospray ionization mass spectrometry (nano-ESI-MS). The observed molecular masses showed discrepancies compared to the calculated molecular masses using the published amino acid sequences. The number of cysteine residues in...
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PMID: 18088070
PDF is available here.
PDF is available here.
Abstract
We report here the application of high resolution Fourier transform ion cyclotron resonance (FT-ICR) mass spectrometry to the characterization of processing intermediates of hydrophobic pulmonary surfactant proteins SP-B and SP-C in intra- alveolar surfactant material of a patient with I73T mutation...
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PMID: 19136726
PDF is available here.
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.
PDF is available here.
Abstract
SP-B was detected in BAL of all 64 patients, whereas SP-C was found in BAL of all but 3 children; those three BAL fluids had more than 80% neutrophils, and in two patients, who were re-lavaged later, SP-C was then present and the neutrophil count was lower. SP-B was mainly present as a dimer, SP-C a...
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PMID: 18405368
PDF is available here.
PDF is available here.
Journal of Gene Medicine (10)1 2008
Abstract
The results of this study indicate that lentiviral vectors with the human CMV enhancer conferring efficient cell-type-specific gene expression may be useful tools for gene therapy purposes or cell tracing, e.g. to analyze stem cell differentiation in transplantation and co-culture settings.
(c) 2007...
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PMID: 18022932
PDF is available here.
PDF is available here.
Human Gene Therapy (19)1 2008
Abstract
We have constructed lentiviral vectors enabling AT2-restricted transgene expression and identification of stem cell-derived AT2 cells. Lung epithelial cell lines M3E3/C3, H441, RLE-6TN, A549, MLE-12, and MLE-15 were characterized at the molecular and ultrastructural levels to identify cell lines use...
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PMID: 18052721
PDF is available here.
PDF is available here.
Biophysical Chemistry (131)1-3 2007
Abstract
A simple surface equation of state is proposed to describe pi-A isotherms of pulmonary surfactant monolayers. The monolayer is considered as undergoing three characteristic states during the compression: the disordered liquid-expanded (LE) state, the ordered liquid-condensed (LC) state and the colla...
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PMID: 17976898
PDF is available here.
PDF is available here.
Biophysical Journal (93)11 2007
Abstract
We simulated the reversible exchange of material between the monolayer and lipid reservoirs under compression and expansion of the interface. We used a mixture of dipalmitoyl-phosphatidylcholine, palmitoyl-oleoyl-phosphatidylglycerol, cholesterol, and surfactant-associated protein C as a functional...
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PMID: 17704166
PDF is available here.
PDF is available here.
Abstract
These results support the potential of developing highly active and inhibition-resistant synthetic exogenous surfactants containing DEPN-8 + apoprotein/peptide constituents for use in treating direct pulmonary forms of clinical acute lung injury (ALI) and the acute respiratory distress syndrome (ARD...
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PMID: 17556674
PDF is available here.
PDF is available here.
Abstract
We constructed a series of lentiviral vectors with regulatory elements predicted to produce lung-specific transgene expression: the surfactant protein C promoter (SPC) for alveolar epithelial type II cell (AECII) expression, the Clara cell 10-kD protein (CC10) for Clara cell expression in the airway...
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PMID: 17575080
PDF is available here.
PDF is available here.
Pediatric Research (62)3 2007
Abstract
Our findings showed that SB rabbits increased their expression of SP-A mRNA and protein after birth compared with NV controls. MV significantly attenuated this response in the absence of dex. Exposure to dex elevated SP-B mRNA expression in both SB and MV rabbits. KGF protein levels were markedly in...
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PMID: 17622950
PDF is available here.
PDF is available here.
Abstract
We hypothesized that other factor(s) besides TTF-1 might contribute to the modulation of SP-C promoter in hypoxic lung cells. Pleomorphic adenoma gene like-2 (PLAGL2), a previously identified TTF-1-independent activator of the SP-C promoter, was not down-regulated, nor increased, within those cells....
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PMID: 17618602
PDF is available here.
PDF is available here.
Abstract
We have reported that in vitro expression of one of these proteins, the exon 4 deletion mutant (hSP-C(Deltaexon4)), causes endoplasmic reticulum (ER) stress, inhibits proteasome function, and activates caspase-3-mediated apoptosis. To further elucidate mechanisms and common pathways for cellular dys...
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PMID: 17586700
PDF is available here.
PDF is available here.
Pediatric Research (62)2 2007
Abstract
We sequenced ABCA3 from four symptomatic infants with the same SFTPC mutation, a substitution of isoleucine by threonine in codon 73 (I73T). Each infant developed respiratory symptoms by 2 mo of age and inherited the mutation from an asymptomatic parent. Three of the four infants were also heterozyg...
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PMID: 17597647
PDF is available here.
PDF is available here.
Immunogenetics (59)7 2007
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.
PDF is available here.
Abstract
Maximum-likelihood models of codon and amino acid substitution were used to analyze the lung-specific surfactant protein C (SP-C) from terrestrial, semi-aquatic, and diving mammals to identify lineages and amino acid sites under positive selection. Site models used the nonsynonymous/synonymous rate...
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PMID: 17568982
PDF is available here.
PDF is available here.
Journal of Pediatrics (150)6 2007
Abstract
Genetic abnormalities of pulmonary surfactant were identified by DNA sequence analysis in 14 (12 full-term, 2 preterm) of 17 newborn infants with fatal respiratory distress of unknown etiology. Deficiency of adenosine triphosphate-binding cassette protein, member A3 (n = 12) was a more frequent caus...
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PMID: 17517255
PDF is available here.
PDF is available here.
Abstract
A crucial aspect of developing a functional, biomimetic lung surfactant (LS) replacement is the selection of the synthetic lipid mixture and surfactant proteins (SPs) or suitable mimics thereof. Studies elucidating the roles of different lipids and surfactant proteins in natural LS have provided cri...
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PMID: 17287113
PDF is available here.
PDF is available here.
Alveolar type II cells isolated from pulmonary adenocarcinoma: a model for JSRV expression in vitro.
Abstract
We developed an in vitro experimental model in which lung epithelial cells were isolated from OPA and control lungs. Cells in culture expressed alveolar type II cell specific markers such as surfactant protein (SP)-A, SP-C, and a high alkaline phosphatase activity. Alveolar Type II cells derived fro...
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PMID: 17158359
PDF is available here.
PDF is available here.
Abstract
We previously demonstrated that 24% of all mouse embryonic stem cell (mESC) derivatives cocultured with embryonic day 11.5 (E11.5) mouse lung rudiments display immunoreactivity to the pneumonocyte II specific marker surfactant-associated protein C (Sftpc). Here we further investigate the effects of...
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PMID: 17220377
PDF is available here.
PDF is available here.
European Biophysics Journal (36)4-5 2007
Abstract
Surfactant protein C (SP-C) is known to be essential for lung function and the formation of a surface confined reservoir at the alveolar interface. The structural features relevant for the peptide's extraordinary ability to form extended three-dimensional structures were systematically investigated...
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PMID: 17051367
PDF is available here.
PDF is available here.
Abstract
We demonstrate that SP-A, SP-B, and SP-D are expressed in human nasal mucosa and cultured NHNE cells. Further study of the functional role of SPs in the upper airway is required....
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PMID: 17209137
PDF is available here.
PDF is available here.
Abstract
We treated a SCLC cell line H526 with a differentiation inducing agent 5-bromodeoxyuridine (BrdU). The treatment led to a dramatic conversion from suspension cells to adherent cells exhibiting an epithelioid phenotype, which remarkably reduced the ability of colony formation in soft agar and suppres...
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PMID: 17196172
PDF is available here.
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.
PDF is available here.
Abstract
We investigated the function of CCTalpha in lung development, using an inducible, epithelial cell-specific CCTalpha knockout mouse line. Deletion of CCTalpha beginning at embryonic day 7.5 did not restrict lung development but resulted in severe respiratory failure at birth. Alveolar lavage and lung...
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PMID: 17130238
PDF is available here.
PDF is available here.
Biochimica et Biophysica Acta (1768)2 2007
Abstract
Pharmacy-grade exogenous lung surfactant preparations of bovine and porcine origin, dispersed in physiological electrolyte solution have been studied. The organization and dynamics at the air/water interface at physiological temperature was analysed by neutron reflection. The results show that a wel...
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PMID: 17156743
PDF is available here.
PDF is available here.