To see the other types of publications on this topic, follow the link: Cystic fibrosis Lungs Life (Biology) Biotechnology.
Journal articles on the topic 'Cystic fibrosis Lungs Life (Biology) Biotechnology'
Create a spot-on reference in APA, MLA, Chicago, Harvard, and other styles
Consult the top 15 journal articles for your research on the topic 'Cystic fibrosis Lungs Life (Biology) Biotechnology.'
Next to every source in the list of references, there is an 'Add to bibliography' button. Press on it, and we will generate automatically the bibliographic reference to the chosen work in the citation style you need: APA, MLA, Harvard, Chicago, Vancouver, etc.
You can also download the full text of the academic publication as pdf and read online its abstract whenever available in the metadata.
Browse journal articles on a wide variety of disciplines and organise your bibliography correctly.
1
Phillipson, Greg. "Cystic fibrosis and reproduction." Reproduction, Fertility and Development 10, no. 1 (1998): 113. http://dx.doi.org/10.1071/r98044.
Full textAbstract:
Cystic fibrosis (CF) is the most common autosomal recessive disease. CF and congenital bilateral absence of the vas deferens (CBAVD) share a genetic and embryological background. Since the 1960s, medical therapy to reduce the progressive obstructive lung disease and nutritional deficiencies has resulted in most CF patients reaching adulthood. With the improved life expectancy of CF patients, new issues in reproductive health and pregnancy management have arisen. Puberty is delayed, with menarche often occurring eighteen months later than the average. Almost all men with CF are azoospermic. In both CF and CBAVD, the vas deferens is absent and the seminal vesicles are often hypoplastic. Many women with CF are subfertile, and if pregnancy is achieved there is an observed increase in maternal morbidity and mortality. The understanding of the molecular basis of CF and CBAVD has evolved, with the identification of hundreds of CF gene mutations and discovery of an associated intron polymorphism of the CF gene. The concept of severe and mild mutations has been introduced to explain the severe and mild phenotype variations such as the pancreatic insufficient and pancreatic sufficient patient. This paper reviews the above issues to assist with the management of infertile couples with CF or CBAVD.
2
Daniels, Jonathan B., Jessica Scoffield, Jessica L. Woolnough, and Laura Silo-Suh. "Impact of glycerol-3-phosphate dehydrogenase on virulence factor production byPseudomonas aeruginosa." Canadian Journal of Microbiology 60, no. 12 (December 2014): 857–63. http://dx.doi.org/10.1139/cjm-2014-0485.
Full textAbstract:
Pseudomonas aeruginosa establishes life-long chronic infections in the cystic fibrosis (CF) lung by utilizing various adaptation strategies. Some of these strategies include altering metabolic pathways to utilize readily available nutrients present in the host environment. The airway sputum contains various host-derived nutrients that can be utilized by P. aeruginosa, including phosphatidylcholine, a major component of lung surfactant. Pseudomonas aeruginosa can degrade phosphatidylcholine to glycerol and fatty acids to increase the availability of usable carbon sources in the CF lung. In this study, we show that some CF-adapted P. aeruginosa isolates utilize glycerol more efficiently as a carbon source than nonadapted isolates. Furthermore, a mutation in a gene required for glycerol utilization impacts the production of several virulence factors in both acute and chronic isolates of P. aeruginosa. Taken together, the results suggest that interference with this metabolic pathway may have potential therapeutic benefits.
3
Lara-Reyna, Samuel, Jonathan Holbrook, Heledd H. Jarosz-Griffiths, Daniel Peckham, and Michael F. McDermott. "Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations." Cellular and Molecular Life Sciences 77, no. 22 (May 4, 2020): 4485–503. http://dx.doi.org/10.1007/s00018-020-03540-9.
Full textAbstract:
Abstract Cystic fibrosis (CF) is one of the most common life-limiting recessive genetic disorders in Caucasians, caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR). CF is a multi-organ disease that involves the lungs, pancreas, sweat glands, digestive and reproductive systems and several other tissues. This debilitating condition is associated with recurrent lower respiratory tract bacterial and viral infections, as well as inflammatory complications that may eventually lead to pulmonary failure. Immune cells play a crucial role in protecting the organs against opportunistic infections and also in the regulation of tissue homeostasis. Innate immune cells are generally affected by CFTR mutations in patients with CF, leading to dysregulation of several cellular signalling pathways that are in continuous use by these cells to elicit a proper immune response. There is substantial evidence to show that airway epithelial cells, neutrophils, monocytes and macrophages all contribute to the pathogenesis of CF, underlying the importance of the CFTR in innate immune responses. The goal of this review is to put into context the important role of the CFTR in different innate immune cells and how CFTR dysfunction contributes to the pathogenesis of CF, highlighting several signalling pathways that may be dysregulated in cells with CFTR mutations.
4
Tomlin, Kerry L., Oisin P. Coll, and Howard Ceri. "Interspecies biofilms ofPseudomonas aeruginosaandBurkholderia cepacia." Canadian Journal of Microbiology 47, no. 10 (October 1, 2001): 949–54. http://dx.doi.org/10.1139/w01-095.
Full textAbstract:
The leading cause of morbidity and mortality in cystic fibrosis (CF) continues to be lung infections with Pseudomonas aeruginosa biofilms. Co-colonization of the lungs with P. aeruginosa and Burkholderia cepacia can result in more severe pulmonary disease than P. aeruginosa alone. The interactions between P. aeruginosa biofilms and B. cepacia are not yet understood; one possible association being that mixed species biofilm formation may be part of the interspecies relationship. Using the Calgary Biofilm Device (CBD), members of all genomovars of the B. cepacia complex were shown to form biofilms, including those isolated from CF lungs. Mixed species biofilm formation between CF isolates of P. aeruginosa and B. cepacia was readily achieved using the CBD. Oxidationfermentation lactose agar was adapted as a differential agar to monitor mixed biofilm composition. Scanning electron micrographs of the biofilms demonstrated that both species readily integrated in close association in the biofilm structure. Pseudomonas aeruginosa laboratory strain PAO1, however, inhibited mixed biofilm formation of both CF isolates and environmental strains of the B. cepacia complex. Characterization of the soluble inhibitor suggested pyocyanin as the active compound.Key words: Burkholderia cepacia, Pseudomonas aeruginosa, mixed biofilms, cystic fibrosis, pyocyanin.
5
Cardenas, Silvia, Mario Scuri, Lennie Samsell, Barbara Ducatman, Pablo Bejarano, Alexander Auais, Melissa Doud, Kalai Mathee, and Giovanni Piedimonte. "Neurotrophic and neuroimmune responses to early-life Pseudomonas aeruginosa infection in rat lungs." American Journal of Physiology-Lung Cellular and Molecular Physiology 299, no. 3 (September 2010): L334—L344. http://dx.doi.org/10.1152/ajplung.00017.2010.
Full textAbstract:
Early-life respiratory infection with Pseudomonas aeruginosa is common in children with cystic fibrosis or immune deficits. Although many of its clinical manifestations involve neural reflexes, little information is available on the peripheral nervous system of infected airways. This study sought to determine whether early-life infection triggers a neurogenic-mediated immunoinflammatory response, the mechanisms of this response, and its relationship with other immunoinflammatory pathways. Weanling and adult rats were inoculated with suspensions containing P. aeruginosa (PAO1) coated on alginate microspheres suspended in Tris-CaCl2 buffer. Five days after infection, rats were injected with capsaicin to stimulate nociceptive nerves in the airway mucosa, and microvascular permeability was measured using Evans blue as a tracer. PAO1 increased neurogenic inflammation in the extra- and intrapulmonary compartments of weanlings but not in adults. The mechanism involves selective overexpression of NGF, which is critical for the local increase in microvascular permeability and for the infiltration of polymorphonuclear leukocytes into infected lung parenchyma. These effects are mediated in part by induction of downstream inflammatory cytokines and chemokines, especially IL-1β, IL-18, and leptin. Our data suggest that neurogenic-mediated immunoinflammatory mechanisms play important roles in airway inflammation and hyperreactivity associated with P. aeruginosa when infection occurs early in life.
6
Ajonuma, Louis Chukwuemeka, Lai Ling Tsang, Gui Hong Zhang, Connie Hau Yan Wong, Miu Ching Lau, Lok Sze Ho, Dewi Kenneth Rowlands, et al. "Estrogen-Induced Abnormally High Cystic Fibrosis Transmembrane Conductance Regulator Expression Results in Ovarian Hyperstimulation Syndrome." Molecular Endocrinology 19, no. 12 (December 1, 2005): 3038–44. http://dx.doi.org/10.1210/me.2005-0114.
Full textAbstract:
Abstract Ovarian hyperstimulation syndrome (OHSS) remains one of the most life-threatening and potentially fatal complications of assisted reproduction treatments, arising from excessive stimulation of the ovaries by exogenous gonadotropins administrated during in vitro fertilization procedures, which is characterized by massive fluid shift and accumulation in the peritoneal cavity and other organs, including the lungs and the reproductive tract. The pathogenesis of OHSS remains obscure, and no definitive treatments are currently available. Using RT-PCR, Western blot, and electrophysiological techniques we show that cystic fibrosis transmembrane conductance regulator (CFTR), a cAMP-activated chloride channel expressed in many epithelia, is involved in the pathogenesis of OHSS. Upon ovarian hyperstimulation, rats develop OHSS symptoms, with up-regulated CFTR expression and enhanced CFTR channel activity, which can also be mimicked by administration of estrogen, but not progesterone, alone in ovariectomized rats. Administration of progesterone that suppresses CFTR expression or antiserum against CFTR to OHSS animals results in alleviation of the symptoms. Furthermore, ovarian hyperstimulation does not induce detectable OHSS symptoms in CFTR mutant mice. These findings confirm a critical role of CFTR in the pathogenesis of OHSS and may provide grounds for better assisted reproduction treatment strategy to reduce the risk of OHSS and improve in vitro fertilization outcome.
7
Lewenza, Shawn, Michelle B. Visser, and Pamela A. Sokol. "Interspecies communication betweenBurkholderia cepaciaandPseudomonas aeruginosa." Canadian Journal of Microbiology 48, no. 8 (August 1, 2002): 707–16. http://dx.doi.org/10.1139/w02-068.
Full textAbstract:
Burkholderia cepacia and Pseudomonas aeruginosa are opportunistic pathogens that commonly cause pulmonary infections in cystic fibrosis patients and occasionally co-infect patients' lungs. Both organisms possess quorum-sensing systems dependent on N-acyl homoserine lactone (N-acyl-HSL). Cross-feeding assays demonstrated that P. aeruginosa and B. cepacia were able to utilize heterologous N-acyl-HSL signaling molecules. The ability of quorum-sensing genes from one species to complement the respective quorum-sensing mutations in the heterologous species was also examined. These studies suggest that B. cepacia CepR can use N-acyl-HSLs synthesized by RhlI and LasI and that P. aeruginosa LasR and RhlR can use N-acyl-HSLs synthesized by CepI. It is possible that a mixed bacterial population of B. cepacia and P. aeruginosa can coordinately regulate some of their virulence factors and influence the progression of lung disease due to infection with these organisms.Key words: quorum sensing, Burkholderia cepacia, Pseudomonas aeruginosa, cystic fibrosis.
8
Beaudoin, Trevor, Shawn D. Aaron, Tracy Giesbrecht-Lewis, Kathy Vandemheen, and Thien-Fah Mah. "Characterization of clonal strains of Pseudomonas aeruginosa isolated from cystic fibrosis patients in Ontario, Canada." Canadian Journal of Microbiology 56, no. 7 (July 2010): 548–57. http://dx.doi.org/10.1139/w10-043.
Full textAbstract:
Pseudomonas aeruginosa is an opportunistic pathogen that can form biofilms in the lungs and airways of cystic fibrosis (CF) patients, resulting in chronic endobronchial infection. Two clonal strains of P. aeruginosa, named type A and type B, have recently been identified and have been found to infect more than 20% of CF patients in Ontario, Canada. In this study, 4 type A and 4 type B isolates retrieved from 8 CF patients in Ontario, Canada, were characterized. All 8 isolates grew well in rich medium and formed biofilms in vitro. Antibiotic resistance profiles of bacteria grown in biofilms and planktonic culture were studied via minimal bactericidal concentration assays for tobramycin, gentamicin, and ciprofloxacin. Compared to laboratory strains of P. aeruginosa, all 8 isolates showed increased resistance to all antibiotics studied in both biofilm and planktonic assays. Gene expression analysis of mexX, representing the MexXY–OprM efflux pump, and mexA, representing MexAB–OprM, revealed that these genes were up-regulated in the 8 clinical isolates. These results suggest clonal type A and type B isolates of P. aeruginosa isolated from CF patients in Ontario, Canada, show a multidrug resistance pattern that can be partially explained as being due to the increased expression of common antibiotic efflux systems.
9
Vincent, Antony T., Steve J. Charette, and Jean Barbeau. "Unexpected diversity in the mobilome of a Pseudomonas aeruginosa strain isolated from a dental unit waterline revealed by SMRT Sequencing." Genome 61, no. 5 (May 2018): 359–65. http://dx.doi.org/10.1139/gen-2017-0239.
Full textAbstract:
The Gram-negative bacterium Pseudomonas aeruginosa is found in several habitats, both natural and human-made, and is particularly known for its recurrent presence as a pathogen in the lungs of patients suffering from cystic fibrosis, a genetic disease. Given its clinical importance, several major studies have investigated the genomic adaptation of P. aeruginosa in lungs and its transition as acute infections become chronic. However, our knowledge about the diversity and adaptation of the P. aeruginosa genome to non-clinical environments is still fragmentary, in part due to the lack of accurate reference genomes of strains from the numerous environments colonized by the bacterium. Here, we used PacBio long-read technology to sequence the genome of PPF-1, a strain of P. aeruginosa isolated from a dental unit waterline. Generating this closed genome was an opportunity to investigate genomic features that are difficult to accurately study in a draft genome (contigs state). It was possible to shed light on putative genomic islands, some shared with other reference genomes, new prophages, and the complete content of insertion sequences. In addition, four different group II introns were also found, including two characterized here and not listed in the specialized group II intron database.
10
Gilleland Jr., H. E. "Adaptive alterations in the outer membrane of gram-negative bacteria during human infection." Canadian Journal of Microbiology 34, no. 4 (April 1, 1988): 499–502. http://dx.doi.org/10.1139/m88-085.
Full textAbstract:
The outer membrane of gram-negative bacteria is a dynamic structure that is capable of altering its ultrastructure and chemistry in order to adapt to changes in its environment. In human infections, outer-membrane alterations are known to play a role in mediating serum resistance, iron uptake, adaptation by Pseudomonas aeruginosa to colonization of the lungs of cystic fibrosis patients, and adaptive resistance to the polymyxin and aminoglycoside antibiotics. This adaptive antibiotic resistance is due to alterations in the cation binding sites within the outer membrane so that these cationic antibiotics can no longer penetrate through the membrane effectively. Adaptive resistance is not stable but is maintained only in the continued presence of the antibiotic. Hence, the role that this type of resistance to cationic antibiotics plays in clinical treatment of human infections remains inadequately assessed.
11
Adlakha, Suneera, Ankur Sharma, Kalpesh Vaghasiya, Eupa Ray, and Rahul Kumar Verma. "Inhalation Delivery of Host Defense Peptides (HDP) using Nano- Formulation Strategies: A Pragmatic Approach for Therapy of Pulmonary Ailments." Current Protein & Peptide Science 21, no. 4 (April 29, 2020): 369–78. http://dx.doi.org/10.2174/1389203721666191231110453.
Full textAbstract:
Host defense peptides (HDP) are small cationic molecules released by the immune systems of the body, having multidimensional properties including anti-inflammatory, anticancer, antimicrobial and immune-modulatory activity. These molecules gained importance due to their broad-spectrum pharmacological activities, and hence being actively investigated. Presently, respiratory infections represent a major global health problem, and HDP has an enormous potential to be used as an alternative therapeutics against respiratory infections and related inflammatory ailments. Because of their short half-life, protease sensitivity, poor pharmacokinetics, and first-pass metabolism, it is challenging to deliver HDP as such inside the physiological system in a controlled way by conventional delivery systems. Many HDPs are efficacious only at practically high molar-concentrations, which is not convincing for the development of drug regimen due to their intrinsic detrimental effects. To avail the efficacy of HDP in pulmonary diseases, it is essential to deliver an appropriate payload into the targeted site of lungs. Inhalable HDP can be a potentially suitable alternative for various lung disorders including tuberculosis, Cystic fibrosis, Pneumonia, Lung cancer, and others as they are active against resistant microbes and cells and exhibit improved targeting with reduced adverse effects. In this review, we give an overview of the pharmacological efficacy of HDP and deliberate strategies for designing inhalable formulations for enhanced activity and issues related to their clinical implications.
12
Picher, Maryse, Lauranell H. Burch, and Richard C. Boucher. "Metabolism of P2 Receptor Agonists in Human Airways." Journal of Biological Chemistry 279, no. 19 (March 1, 2004): 20234–41. http://dx.doi.org/10.1074/jbc.m400305200.
Full textAbstract:
Extracellular nucleotides are among the most potent mediators of mucociliary clearance (MCC) in human lungs. However, clinical trials revealed that aerosolized nucleotides provide only a transient improvement of MCC to patients diagnosed with cystic fibrosis (CF). In this study, we identified the mechanism that eliminates extracellular nucleotides from human airways. Polarized primary cultures of human bronchial epithelial cells were impermeable to extracellular nucleotides but rapidly dephosphorylated ATP into ADP, AMP, and adenosine. The half-life of a therapeutic ATP concentration (0.1 mm) was ∼20 s within the periciliary liquid layer. The mucosal epithelial surface eliminated P2 receptor agonists (ATP = UTP > ADP > UDP) at 3-fold higher rates than the serosal surface. We also showed that mucosal (not serosal) ectoATPase activity increases toward areas most susceptible to airway obstruction (nose < bronchi << bronchioles). Bronchial cultures from patients with CF, primary ciliary dyskinesia, or α1-antitrypsin deficiency exhibited 3-fold higher mucosal (not serosal) ectoATPase activity than normal cultures. Time course experiments indicated that CF enhances ATP elimination and adenosine accumulation on the mucosal surface. Furthermore, nonspecific alkaline phosphatase was identified as the major regulator of airway nucleotide concentrations in CF, primary ciliary dyskinesia, and α1-antitrypsin deficiency. The ectoAT-Pase activity and mRNA expression of mucosally restricted nonspecific alkaline phosphatase were 3-fold higher on bronchial cultures from these patients than from healthy subjects. This study demonstrates that the duration of nucleotide-mediated MCC is limited by epithelial ectonucleotidases throughout human airways, with the efficiency of this mechanism enhanced in chronic inflammatory lung diseases, including CF.
13
Cigana, Cristina, Nicola Ivan Lorè, Maria Lina Bernardini, and Alessandra Bragonzi. "Dampening Host Sensing and Avoiding Recognition inPseudomonas aeruginosaPneumonia." Journal of Biomedicine and Biotechnology 2011 (2011): 1–10. http://dx.doi.org/10.1155/2011/852513.
Full textAbstract:
Pseudomonas aeruginosais an opportunistic pathogen and causes a wide range of acute and chronic infections.P. aeruginosainfections are kept in check by an effective immune surveillance in the healthy host, while any imbalance or defect in the normal immune response can manifest in disease. Invasive acute infection in the immunocompromised patients is mediated by potent extracellular and cell bound bacterial virulence factors. Life-threatening chronic infection in cystic fibrosis patients is maintained by pathogenic variants that contribute to evade detection and clearance by the immune system. Here, we reviewed the molecular basis of receptor-mediated recognition ofP. aeruginosaand their role in initiating inflammation and the colonization. In addition, the consequence of theP. aeruginosagenetic adaptation for the antibacterial defence and the maintaining of chronic infection are discussed.
14
McNeely, Damian, Ryan M. Chanyi, James S. Dooley, John E. Moore, and Susan F. Koval. "Biocontrol of Burkholderia cepacia complex bacteria and bacterial phytopathogens by Bdellovibrio bacteriovorus." Canadian Journal of Microbiology 63, no. 4 (April 2017): 350–58. http://dx.doi.org/10.1139/cjm-2016-0612.
Full textAbstract:
Bdellovibrio and like organisms are predatory bacteria that have the unusual property of using the cytoplasmic constituents of other Gram-negative bacteria as nutrients. These predators may thus provide an alternative approach to the biocontrol of human and plant pathogens. Predators were isolated on Burkholderia cenocepacia K56-2 and J2315 as prey cells, in enrichment cultures with soil and sewage. Three isolates (DM7C, DM8A, and DM11A) were identified as Bdellovibrio bacteriovorus on the basis of morphology, a periplasmic life cycle, and 16S rRNA gene sequencing. The prey range of these isolates was tested on Burkholderia cepacia complex bacteria and several phytopathogenic bacteria of agricultural importance. Of 31 strains of the Burkholderia cepacia complex tested, only 4 were resistant to predation by strain DM7C. A subset of 9 of the prey tested were also susceptible to strains DM8A and DM11A. Of 12 phytopathogens tested, 4 were resistant to strains DM7C and DM8A, and only 2 were resistant to strain DM11A. Thus, Bdellovibrio bacteriovorus strains retrieved from environmental samples on 2 Burkholderia cenocepacia isolates from cystic fibrosis patients did not distinguish in their prey range between other isolates of that pathogen or phytopathogens. Such strains hold promise as potential wide-spectrum biocontrol agents.
15
Jouault, Albane, Angélique Gobet, Marjolaine Simon, Emilie Portier, Morgan Perennou, Erwan Corre, Fanny Gaillard, et al. "Alterocin, an Antibiofilm Protein Secreted by Pseudoalteromonas sp. Strain 3J6." Applied and Environmental Microbiology 86, no. 20 (August 7, 2020). http://dx.doi.org/10.1128/aem.00893-20.
Full textAbstract:
ABSTRACT We sought to identify and study the antibiofilm protein secreted by the marine bacterium Pseudoalteromonas sp. strain 3J6. The latter is active against marine and terrestrial bacteria, including Pseudomonas aeruginosa clinical strains forming different biofilm types. Several amino acid sequences were obtained from the partially purified antibiofilm protein, named alterocin. The Pseudoalteromonas sp. 3J6 genome was sequenced, and a candidate alt gene was identified by comparing the genome-encoded proteins to the sequences from purified alterocin. Expressing the alt gene in another nonactive Pseudoalteromonas sp. strain, 3J3, demonstrated that it is responsible for the antibiofilm activity. Alterocin is a 139-residue protein that includes a predicted 20-residue signal sequence, which would be cleaved off upon export by the general secretion system. No sequence homology was found between alterocin and proteins of known functions. The alt gene is not part of an operon and adjacent genes do not seem related to alterocin production, immunity, or regulation, suggesting that these functions are not fulfilled by devoted proteins. During growth in liquid medium, the alt mRNA level peaked during the stationary phase. A single promoter was experimentally identified, and several inverted repeats could be binding sites for regulators. alt genes were found in about 30% of the Pseudoalteromonas genomes and in only a few instances of other marine bacteria of the Hahella and Paraglaciecola genera. Comparative genomics yielded the hypothesis that alt gene losses occurred within the Pseudoalteromonas genus. Overall, alterocin is a novel kind of antibiofilm protein of ecological and biotechnological interest. IMPORTANCE Biofilms are microbial communities that develop on solid surfaces or interfaces and are detrimental in a number of fields, including for example food industry, aquaculture, and medicine. In the latter, antibiotics are insufficient to clear biofilm infections, leading to chronic infections such as in the case of infection by Pseudomonas aeruginosa of the lungs of cystic fibrosis patients. Antibiofilm molecules are thus urgently needed to be used in conjunction with conventional antibiotics, as well as in other fields of application, especially if they are environmentally friendly molecules. Here, we describe alterocin, a novel antibiofilm protein secreted by a marine bacterium belonging to the Pseudoalteromonas genus, and its gene. Alterocin homologs were found in about 30% of Pseudoalteromonas strains, indicating that this new family of antibiofilm proteins likely plays an important albeit nonessential function in the biology of these bacteria. This study opens up the possibility of a variety of applications.