• Previous studies have demonstrated that lung-specific proteins are associated with surfactant lipids, particularly the highly surface active subfraction known as tubular myelin. (nih.gov)
  • We have isolated a surfactant-associated protein complex with molecular weight components of 36 000, 32 000, and 28 000 and reassembled it with protein-free lung surfactant lipids prepared as small unilamellar liposomes. (nih.gov)
  • The effects of divalent cations on the structure and surface activity of this protein-lipid mixture were investigated by following (1) the state of lipid dispersion by changes in turbidity and by electron microscopy and (2) the ability of the surfactant lipids to form a surface film from an aqueous subphase at 37 degrees C. The protein complex markedly increased the rate of Ca2+-induced surfactant-lipid aggregation. (nih.gov)
  • Similarly, 5 mM Ca2+ but not 5 mM Mg2+ improved the ability of the protein-lipid mixture to form a surface film at 37 degrees C. Extensive aggregation of the surfactant lipids without protein by 20 mM Ca2+ or 20 mM Mg2+ did not promote rapid surface film formation. (nih.gov)
  • Pulmonary surfactant is mainly composed of phospholipids and minor amounts of other lipids and specific proteins. (downstate.edu)
  • A delicate balance between synthesis, secretion, recycling and degradation of the lipids is required to maintain surfactant function and lung performance. (downstate.edu)
  • Samaha G., Garcia-Arcos I., (2020) "Alveolar lipids in pulmonary disease. (downstate.edu)
  • The saposin B-type domain is a ~80 amino acid domain present in saposins and related proteins that interact with lipids. (embl.de)
  • At this interface, proteins and lipids of the pulmonary surfactant bind to MWCNTs, affecting their surface characteristics. (biomedcentral.com)
  • In preterm infants, surfactant replacement therapies that include lipids and the hydrophobic surfactant proteins are highly efficacious in improving lung function. (fz-borstel.de)
  • Familial pulmonary fibrosis is a heterogeneous group of interstitial lung diseases of unknown cause that is associated with multiple pathologic subsets. (nih.gov)
  • A transversion of SFTPC in a large familial pulmonary fibrosis kindred, including adults with usual interstitial pneumonitis and children with cellular nonspecific interstitial pneumonitis. (nih.gov)
  • We show that an SFTPC mutation segregates with the pulmonary fibrosis phenotype in this kindred and may cause type II cellular injury. (nih.gov)
  • The scientists compared levels of a variety of proteins obtained from the lung tissues of healthy individuals, patients with COPD, and those with pulmonary fibrosis. (sciencedaily.com)
  • Targeted AntiBiotics for Chronic pulmonary diseases (TARGET ABC): can targeted antibiotic therapy improve the prognosis of Pseudomonas aeruginosa-infected patients with chronic pulmonary obstructive disease, non-cystic fibrosis bronchiectasis, and asthma? (sdu.dk)
  • Genetic variants associated with susceptibility to idiopathic pulmonary fibrosis in people of European ancestry: a genome-wide association study. (nature.com)
  • Single-cell deconvolution of fibroblast heterogeneity in mouse pulmonary fibrosis. (nature.com)
  • Single-cell transcriptomic analysis of human lung provides insights into the pathobiology of pulmonary fibrosis. (nature.com)
  • Single-cell RNA sequencing identifies diverse roles of epithelial cells in idiopathic pulmonary fibrosis. (nature.com)
  • We evaluated whether lysyl oxidase-like 2 (LOXL2), which promotes cross-linking of collagen in pathological stroma, was detectable in serum from idiopathic pulmonary fibrosis (IPF) patients, and assessed its relationship with IPF disease progression. (ersjournals.com)
  • Idiopathic pulmonary fibrosis (IPF) is a progressive and ultimately fatal disease of the lungs involving airway epithelial cell damage, fibroblast activation and proliferation, and excessive deposition of collagen and other extracellular matrix (ECM) components [ 1 ]. (ersjournals.com)
  • PD-L1 on invasive fibroblasts drives fibrosis in a humanized model of idiopathic pulmonary fibrosis. (cedars-sinai.edu)
  • Hyaluronan and TLR4 promote surfactant-protein-C-positive alveolar progenitor cell renewal and prevent severe pulmonary fibrosis in mice. (cedars-sinai.edu)
  • This condition results from abnormalities in the composition or function of surfactant, a mixture of certain fats (called phospholipids) and proteins that lines the lung tissue and makes breathing easy. (medlineplus.gov)
  • The phospholipids and proteins that make up surfactant are packaged in cellular structures known as lamellar bodies. (medlineplus.gov)
  • Surfactant is composed of phospholipids and other surfactant-associated proteins. (avivasysbio.com)
  • These include the generation of a variety of lipid mediators, along with membrane remodelling, modification of extracellular non-cellular phospholipid components of pulmonary surfactant, microparticles and lipoproteins, and degradation of microbial membranes and dietary phospholipids. (ijbs.com)
  • Description: A sandwich ELISA kit for detection of Surfactant Associated Protein C from Rat in samples from blood, serum, plasma, cell culture fluid and other biological fluids. (stemcellcharter.org)
  • Idiopathic persistent pulmonary hypertension of the newborn can present without signs of acute perinatal distress. (medscape.com)
  • Without normal surfactant, the tissue surrounding the air sacs in the lungs (the alveoli) sticks together (because of a force called surface tension) after exhalation, causing the alveoli to collapse. (medlineplus.gov)
  • These two proteins help spread the surfactant across the surface of the lung tissue, aiding in the surface tension-lowering property of surfactant. (medlineplus.gov)
  • The loss of functional surfactant raises surface tension in the alveoli, causing severe breathing problems. (medlineplus.gov)
  • Pulmonary surfactant is a lipid-rich material that prevents lung collapse by lowering surface tension at the air-liquid interface in the alveoli of lung. (avivasysbio.com)
  • The major biophysical function of this lung surfactant film is to reduce the alveolar surface tension to near-zero value. (biophysics.org)
  • Surfactants are compounds that lower the surface tension of a liquid, the interfacial tension between two liquids, or that between a liquid and a solid. (absoluteastronomy.com)
  • It is also the only surface active component of lung surfactant capable of lowering surface tension to near zero levels. (absoluteastronomy.com)
  • SP-A is covered on the alveolus surface to decrease the surface tension, thus prevent alveolus collapse, and stabilize pulmonary alveolar pressure [ 9 ]. (hindawi.com)
  • Surfactant allows the lungs to inflate by decreasing the alveolar surface tension and the work needed for inspiration in each breathing cycle. (downstate.edu)
  • Type II cells secrete pulmonary surfactant (a phospholipid bound to a protein) that reduces the surface tension of the moisture that covers the alveolar walls. (cliffsnotes.com)
  • We also show the presence of normal lamellar bodies and the secretion of surfactant into the cell culture medium in the organoids of lentiviral infected cells. (nature.com)
  • Effects on pulmonary surfactant secretion were assessed through changes in phospholipid content of the lavagate. (cdc.gov)
  • Phosgene also caused significant increases in alveolar/capillary membrane permeability immediately after exposure and significant increases in pulmonary surfactant secretion 24 to 26 hours postexposure. (cdc.gov)
  • Adaptation to air breathing after birth is dependent upon the synthesis and secretion of pulmonary surfactant by alveolar type 2 (AT2) cells. (stanford.edu)
  • Alveolar type II (ATII) epithelial cells synthesize, secrete and recycle all components of surfactant and dysfunction in surfactant metabolism can result in a variety of pediatric lung diseases including respiratory distress syndrome and interstitial lung disease 3 . (nature.com)
  • Dr. Garcia-Arcos leads a team of scientists focused on lipid metabolism, surfactant lipid homeostasis, and cellular lipid uptake and usage during pulmonary and cardiovascular disease. (downstate.edu)
  • While it has been known for decades that an adequate development of the alveolar surfactant system is essential for neonates, the role of lipid metabolism in adult lung disease is poorly understood despite accumulating evidence showing its relevance. (downstate.edu)
  • Pulmonary metabolism in smoke-related disease. (downstate.edu)
  • Surfactant protein B (SFTPB) deficiency is a fatal disease affecting newborn infants. (nature.com)
  • After differentiating the mutant and corrected cells into lung organoids, we show expression of SFTPB mRNA during endodermal and organoid differentiation but the protein product only after organoid differentiation. (nature.com)
  • Surfactant dysfunction is caused by mutations in one of several genes, including SFTPB , SFTPC , and ABCA3 . (medlineplus.gov)
  • The SFTPB and SFTPC genes provide instructions for making surfactant protein-B (SP-B) and surfactant protein-C (SP-C), respectively, two of the four proteins in surfactant. (medlineplus.gov)
  • Mutations in the SFTPB gene cause a type of surfactant dysfunction sometimes referred to as SP-B deficiency. (medlineplus.gov)
  • These mutations lead to a reduction in or absence of mature SP-B. In addition, SFTPB gene mutations cause abnormal processing of SP-C, resulting in a lack of mature SP-C and a buildup of unprocessed forms of SP-C. These changes lead to abnormal surfactant composition and decreased surfactant function. (medlineplus.gov)
  • SFTPB is the pulmonary-associated surfactant B protein (SPB), an amphipathic surfactant protein essential for lung function and homeostasis after birth. (avivasysbio.com)
  • Next are patients with genetic mutations in: surfactant protein B (SFTPB), ABCA3, surfactant protein C (SFTPC) and pulmonary alveolar proteinosis (PAP). (cincinnatichildrens.org)
  • Dipalmitoylphosphatidylcholine is a phospholipid consisting of two palmitic acids and is the major constituent of pulmonary surfactant. (absoluteastronomy.com)
  • SP-D has a significant roles in immune and inflammatory regulation of the lung as it regulates of the level of surfactant in the lungs by a process named surfactant homeostasis. (wikipedia.org)
  • In this study, we tested the possibility that CDH lungs are surfactant protein deficient, which could explain the respiratory failure and difficulties in treating CDH infants. (eur.nl)
  • Immunohistochemical analysis revealed no differences between control and CDH lungs for SP protein expression. (eur.nl)
  • Pulmonary function tests and a carbon monoxide diffusion capacity test of the lungs for carbon monoxide (DLCO) may be performed. (medscape.com)
  • Pulmonary alveolar proteinosis is a rare disorder in which the air sacs of the lungs (alveoli) become plugged with a protein- and fat-rich fluid. (msdmanuals.com)
  • The cause of pulmonary alveolar proteinosis is almost always unknown, but studies have linked it to production of an antibody directed against a protein that seems to be involved with the production or the breakdown of surfactant (a substance normally produced in the lungs). (msdmanuals.com)
  • The protein in the lungs plugs up the alveoli and small airways (bronchioles). (msdmanuals.com)
  • Dr. Ledford's current work in the area of pulmonary surfactant immunobiology combines her knowledge of mouse genetics, pulmonary disease models and immune function regulation and focuses on understanding the role of Surfactant Protein-A (SP-A) and how it regulates signaling pathways within various immune cell populations. (bio5.org)
  • Mutations in the surfactant protein C (SP-C) gene (SFTPC) are associated with familial desquamative and nonspecific interstitial pneumonitis. (nih.gov)
  • Mutations in the SFTPC gene are involved in a type of surfactant dysfunction sometimes called SP-C dysfunction. (medlineplus.gov)
  • Neonates with congenital diaphragmatic hernia (CDH) suffer from a diaphragmatic defect, lung hypoplasia, and pulmonary hypertension, with poor lung function forming the major clinical challenge. (eur.nl)
  • Dr. Pryhuber's particular clinical interests are care of infants with pulmonary hypertension, lung injury, growth and developmental failure, often complicated by chronic and inflammatory disease. (rochester.edu)
  • She leads the neonatal pulmonary hypertension consultation team. (rochester.edu)
  • Persistent pulmonary hypertension of the newborn (PPHN) is defined as the failure of the normal circulatory transition that occurs after birth. (medscape.com)
  • It is a syndrome characterized by marked pulmonary hypertension that causes hypoxemia secondary to right-to-left shunting of blood at the foramen ovale and ductus arteriosus. (medscape.com)
  • In contrast to adult primary pulmonary hypertension, the newborn syndrome is not defined by a specific pressure of the pulmonary circulation. (medscape.com)
  • Patients with DLCO of less than 60% predicted and oxygen desaturation of less than 90% on the 6-minute walk test have a high likelihood of pulmonary hypertension and should undergo further evaluation for the presence of this disorder. (medscape.com)
  • [ 51 ] In one study, patients with a saturation of less than 90% during a 6-minute walk test were 12 times more likely to have pulmonary hypertension. (medscape.com)
  • To determine the correlation between maternal bile acid (BA) level and fetal pulmonary surfactant in rats and study the effects of BA on fetal lung in rat model of intrahepatic cholestasis of pregnancy. (hindawi.com)
  • Deletion of the glucocorticoid receptor gene (Nr3c1) from fetal pulmonary mesenchymal cells phenocopied defects caused by global Nr3c1 deletion, while lung epithelial- or endothelial-specific Nr3c1 deletion did not impair lung function at birth. (stanford.edu)
  • Mar. 10, 2023 One in 10 adults suffer from the debilitating effects of chronic obstructive pulmonary disease (COPD). (sciencedaily.com)
  • Surfactant is produced by alveolar type II cells which can be differentiated in vitro from patient specific induced pluripotent stem cell (iPSC)-derived lung organoids. (nature.com)
  • In the lab, we use cell culture and in vivo models to study the regulation of surfactant homeostasis and its dysregulation during disease. (downstate.edu)
  • Congenital PAP is caused by mutations in genes required for normal surfactant production. (medscape.com)
  • These results add to the growing evidence that specific Ca2+-protein-lipid interactions are important in determining both the structure and function of extracellular lung surfactant fractions. (nih.gov)
  • The authors conclude that colchicine may have a potential role in lung injury that is associated with increased permeability or extracellular surfactant. (cdc.gov)
  • We conclude that there is no primary deficiency of surfactant proteins in the nitrofen-induced CDH rat model. (eur.nl)
  • One type of surfactant dysfunction, SP-B deficiency, is estimated to occur in 1 in 1 million newborns worldwide. (medlineplus.gov)
  • For this reason, the skin is often exposed to potentially hazardous agents, including chemicals, which may contribute to the onset of a spectrum of adverse health effects ranging from localized damage (e.g., irritant contact dermatitis and corrosion) to induction of immune-mediated responses (e.g., allergic contact dermatitis and pulmonary responses), or systemic toxicity (e.g., neurotoxicity and hepatoxicity). (cdc.gov)
  • Secondary PAP occurs in a heterogeneous group of conditions that reduce numbers or functions of alveolar macrophages and thereby surfactant clearance. (medscape.com)
  • Aim of the present study was to investigate if the pre-coating of MWCNTs with pulmonary surfactant has an influence on potential adverse effects, upon both (i) human monocyte derived macrophages (MDM) monocultures, and (ii) a sophisticated in vitro model of the human epithelial airway barrier. (biomedcentral.com)
  • The exaggerated immune response to repeated inhalation of these particles leads to infiltration and proliferation of activated pulmonary macrophages and lymphocytes, resulting in lymphocytic alveolitis and bronchiolitis with noncaseating granulomas. (cdc.gov)
  • Each of these genes is involved in the production of surfactant. (medlineplus.gov)
  • Chloroquine has also been shown to be efficacious for the treatment and maintenance of chronic pulmonary sarcoidosis. (medscape.com)
  • Surfactant protein A contains a N-terminal collagen-like domain and a C-terminal lectin domain that are characteristic of members of the collectin family of proteins. (nih.gov)
  • Recently, the abnormalities of pulmonary surfactant system have been implicated in the pathogenesis of respiratory distress syndrome [ 6 , 7 ]. (hindawi.com)
  • Blood test results may show abnormalities that occur in pulmonary alveolar proteinosis as well as in other disorders. (msdmanuals.com)
  • She serves on NIH study sections and as reviewer for journals in her area of expertise, manages an active laboratory, supervises students in pulmonary biology and epithelial cell research and has received awards recognizing both teaching and original research accomplishments. (rochester.edu)
  • The distal lung is a honeycomb-like collection of delicate gas exchange sacs called alveoli lined by two interspersed epithelial cell types: the cuboidal, surfactant-producing alveolar type II (AT2) and the flat, gas-exchanging alveolar type I (AT1) cell. (stanford.edu)
  • Surfactant is released from the lung cells and spreads across the tissue that surrounds alveoli. (medlineplus.gov)
  • Found in the lung parenchyma, the pulmonary alveoli are the dead ends of the respiratory tree, which outcrop from either alveolar sacs or alveolar ducts, which are both sites of gas exchange with the blood as well. (absoluteastronomy.com)
  • It is a condition where the alveoli are deflated, as distinct from pulmonary consolidation.It is a very common finding in chest x-rays and other. (absoluteastronomy.com)
  • In the alveoli, MWCNTs first interact with the pulmonary surfactant. (biomedcentral.com)
  • To make a definitive diagnosis of pulmonary alveolar proteinosis, doctors examine a sample of the fluid from the alveoli. (msdmanuals.com)
  • For example, levels of some substances (for example, lactic dehydrogenase, red blood cells, serum surfactant proteins, and gamma globulin) are often elevated. (msdmanuals.com)
  • Researchers in Finland are reporting identification of the first potential "biomarker" that could be used in development of a sputum test for early detection of chronic obstructive pulmonary disease (COPD). (sciencedaily.com)
  • Background: Welders are exposed to gas and particle emissions that can cause severe lung disease, such as chronic obstructive pulmonary disease (COPD), a leading cause of mortality and morbidity worldwide. (lu.se)
  • SP-A is a major player in the pulmonary cytokine-network and moreover has been described to act in the pulmonary host defense. (iospress.com)
  • The diagnosis is confirmed regardless of the pulmonary arterial pressure, as long as it is accompanied by a right-to-left shunt and absence of congenital heart disease. (medscape.com)
  • We investigated this hypothesis in the nitrofen-induced CDH rat model and assessed the cellular concentrations of surfactant protein (SP)-A, -B, and -C mRNA with a quantitative radioactive in situ hybridization technique. (eur.nl)
  • These misfolded proteins may trigger a cellular response that results in cell damage and death. (medlineplus.gov)
  • SP-A-deficient mice have an enhanced susceptibility to infection to pulmonary infection with bacterial and fungal pathogens and collectin replacement in these animals corrects defects in dysregulated cellular functions and microbial clearance. (fz-borstel.de)
  • Previous reports demonstrate that surfactant protein-A (SP-A) binds live M. pneumoniae and mycoplasma membrane fractions (MMF) with high affinity. (bio5.org)
  • An abundant pulmonary surfactant-associated protein that binds to a variety of lung pathogens, resulting in their opsinization. (nih.gov)
  • Recently, bile acid was found to cause surfactant inactivation by enhancing the activity of secretory phospholipase A2 (sPLA2) and induce inflammatory response of fetal lung [ 5 , 8 ]. (hindawi.com)
  • Deficiencies and inactivation of surfactant have been associated with a variety of human lung diseases in both infants and adults. (fz-borstel.de)
  • We believe the answer is probable, since some of the systemic disease clinical manifestations of COVID-19 cannot be explained solely by the binding of SARS-CoV-2 spike proteins with cell membranes of tissues that exhibit angiotensin-converting enzyme 2 (ACE2). (frontiersin.org)
  • Hypersensitivity pneumonitis, also known as extrinsic allergic alveolitis, is an uncommon non-immunoglobulin E (IgE), T-helper cell type 1 (Th1)-mediated inflam- matory pulmonary disease with systemic symptoms resulting from repeated inhalation and subsequent sensitization to a large variety of aerosolized antigenic organic dust particles. (cdc.gov)
  • We sought to determine whether immune reactivity occurs between anti-SARS-CoV-2 protein antibodies and human tissue antigens, and whether molecular mimicry between COVID-19 viral proteins and human tissues could be the cause. (frontiersin.org)
  • We applied both human monoclonal anti-SARS-Cov-2 antibodies (spike protein, nucleoprotein) and rabbit polyclonal anti-SARS-Cov-2 antibodies (envelope protein, membrane protein) to 55 different tissue antigens. (frontiersin.org)
  • We found that SARS-CoV-2 antibodies had reactions with 28 out of 55 tissue antigens, representing a diversity of tissue groups that included barrier proteins, gastrointestinal, thyroid and neural tissues, and more. (frontiersin.org)
  • In this mechanism, antibodies formed against SARS-CoV-2 would also bind to human tissue proteins leading to autoimmune reactivity. (frontiersin.org)
  • Tests for granulocyte macrophage colony stimulating factor (GM-CSF) antibodies can also be performed to help support the diagnosis of an autoimmune form of pulmonary alveolar proteinosis. (msdmanuals.com)
  • However, whether the level of TBA in maternal serum caused perinatal abnormality of pulmonary surfactant and fetal lung tissue morphological structure remains largely unknown. (hindawi.com)
  • We also did selective epitope mapping using BLAST and showed similarities and homology between spike, nucleoprotein, and many other SARS-CoV-2 proteins with the human tissue antigens mitochondria M2, F-actin and TPO. (frontiersin.org)
  • Cross-reactivity occurs when amino acid sequence homology exists between a pathogen and self-tissue proteins ( 1 ). (frontiersin.org)
  • LOXL2 protein expression is observed in the fibroblastic foci and collagenous regions of diseased IPF lung tissue, with relatively minor expression in healthy lung tissue [ 9 ]. (ersjournals.com)
  • Description: This is Double-antibody Sandwich Enzyme-linked immunosorbent assay for detection of Rat Surfactant Associated Protein C (SPC) in Tissue homogenates, cell lysates, lung lavage fluid and other biological fluids. (stemcellcharter.org)
  • Description: A sandwich quantitative ELISA assay kit for detection of Human Surfactant Associated Protein C (SPC) in samples from serum, plasma, tissue homogenates, cell lysates, lung lavage fluid, cell culture supernates or other biological fluids. (stemcellcharter.org)
  • Neither artificial membrane slabs, nor 'live cells' imaged under conditions in which cells have a shabby life that doesn't last long (how much of this is due to the mistreatment of the membrane proteins? (biophysics.org)
  • As to the role of the cytoskeleton, what does this tell us about the membrane itself, or the behavior of membrane proteins as individual molecules in their interplay with the membrane? (biophysics.org)
  • Lack of mature SP-C can lead to abnormal composition of surfactant and decreased surfactant function. (medlineplus.gov)
  • Air trapping is a common feature in sarcoidosis that can be supported with imaging studies and correlates with evidence of small airways disease on pulmonary function testing. (medscape.com)
  • By the use of cell culture … or animal models the functional properties have been repeatedly shown in many aspects often bearing surprising properties which strongly indicate the physiological importance of SP-A. To date SP-A is recognized as a molecule essential for pulmonary development, structure and function. (iospress.com)
  • However, data suggest early treatment of stage II sarcoidosis with oral prednisolone for 3 months followed by inhaled budesonide for 15 months improves 5-year pulmonary function and reduces the need for future steroid treatment. (medscape.com)
  • This article gives an overview about the state of knowledge on SP-A focussed in applications for human pulmonary disorders and points out the importance for pathology-orientated research approaches using immunohistochemistry or in Situ hybridization as promising methods to further elucidate the role of this molecule in adult lung diseases. (iospress.com)
  • Less severe forms of surfactant dysfunction cause gradual onset of breathing problems in children or adults. (medlineplus.gov)
  • Intratracheally injection of bile acid had been shown to induce severe pulmonary edema in rabbits [ 15 ]. (hindawi.com)
  • An upcoming number of reports deals with the role of SP-A for pulmonary pathology. (iospress.com)
  • In molecular biology, Pulmonary surfactant protein D (SP-D) is a protein domain predominantly found in lung surfactant. (wikipedia.org)
  • These antigens include organic dusts containing bacteria, fungi,4 animal or plant proteins, or low-molecular-weight chemicals. (cdc.gov)
  • Lung measurements were performed with standard spirometry and new methods: airspace dimension assessment (AiDA), oscillometry, blood serum biomarkers (club cell secretory protein 16, surfactant protein D, matrix metalloproteinases, fibroblast, hepatocyte growth factor, interleukins), and one urine biomarker (desmosine). (lu.se)
  • Finally, the further GIX sPLA 2 s are found in venom of marine snails, and GXIA and GXIB sPLA 2 s are plant proteins. (ijbs.com)
  • In one study, treatment of acute exacerbations of pulmonary sarcoidosis with steroid doses as low as 20 mg of prednisone for a median of 21 days improved spirometry back to baseline and improved clinical symptoms. (medscape.com)