Mouse cystic fibrosis transmembrane. Medical search. Frequent questions

Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane protein and anion channel in vertebrates that is encoded by the CFTR gene. (wikipedia.org)
Each individual inherits two copies of the CFTR (cystic fibrosis transmembrane conductance regulator) gene. (wikipedia.org)
Involvement of cystic fibrosis transmembrane conductance regulator in mouse sperm capacitation. (umassmed.edu)
Recognizes a protein of 165-170kDa, identified as cystic fibrosis transmembrane conductance regulator (CFTR). (neobiotechnologies.com)
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene. (academictree.org)
The putative gene in cystic fibrosis (CF) encodes a protein, cystic fibrosis transmembrane conductance regulator ( CFTR ), which has an important role in transepithelial salt transport. (bmj.com)
The Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) gene was identified in 1989 by geneticist Lap-Chee Tsui and his research team as the gene associated with cystic fibrosis (CF). Tsui's research pinpointed the gene, some mutations to which cause CF, and it revealed the underlying disease mechanism. (asu.edu)
Cystic fibrosis (CF) is a genetic disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. (ox.ac.uk)
We asked if murine mucosal defenses and airway surface liquid (ASL) Cl(-) were altered by the DeltaF508 cystic fibrosis transmembrane conductance regulator mutation. (lookformedical.com)
It is caused by mutations of the gene coding for cystic fibrosis transmembrane conductance regulator (CFTR). (eurasiareview.com)
It's known that CF is associated with a protein known as cystic fibrosis transmembrane conductance regulator (CFTR), the production of which is encoded by the CFTR-gene. (sensiseeds.com)
METHODS: For 3 weeks, we administered oral AB (ciprofloxacin/metronidazole) or control treatment to homozygous Delta F508 (Delta/Delta), cystic fibrosis transmembrane conductance regulator (CFTR) knockout (-/-), and wild-type (WT) mice and quantified fat absorption using a 72-h fat balance test. (eur.nl)
BACKGROUND: The cystic fibrosis transmembrane conductance regulator gene (CFTR) shows a complex pattern of expression. (ox.ac.uk)
Here, we show that apoptotic body like liposomes loaded with phosphatidylinositol 5-phosphate (ABL/PI5P) enhance the antimycobacterial response, both in macrophages from healthy donors exposed to pharmacological inhibition of cystic fibrosis transmembrane conductance regulator (CFTR) and in macrophages from CF patients, by enhancing phagosome acidification and reactive oxygen species (ROS) production. (torvergata.it)
This disease is caused by mutations in the cystic fibrosis transmembrane and conductance regulator (CFTR) gene and the resulting of dysfunctional CFTR protein. (uni-tuebingen.de)
Loss of the cystic fibrosis transmembrane conductance regulator (CFTR-/-) in macrophages (mphis) has been associated with lyosomal alkalinization. (stanfordchildrens.org)
Our results, supported by a promising preclinical pharmacological effect of full restoration of cystic fibrosis transmembrane conductance regulator channel activity, emphasize the high potential of SPL84 as an effective drug for the treatment of CF patients. (researcher.life)
Expression of the cystic fibrosis transmembrane conductance regulator gene ( CFTR ) can be detected in the pancreatic ductules at 18 weeks' gestation. (medscape.com)

Geneticist Lap-Chee Tsui and his team identified the CFTR gene in 1989 as the gene linked with CF (cystic fibrosis). (wikipedia.org)
Mutations of the CFTR gene affecting anion channel function lead to dysregulation of epithelial lining fluid (mucus) transport in the lung, pancreas and other organs, resulting in cystic fibrosis. (wikipedia.org)
Cells expressing a mutant form of the CFTR protein are resistant to invasion by the Salmonella typhi bacterium, the agent of typhoid fever, and mice carrying a single copy of mutant CFTR are resistant to diarrhea caused by cholera toxin. (wikipedia.org)
Acquired cystic fibrosis transmembrane regulator (CFTR) dysfunctions have been associated with several conditions, including myocardial infarction (MI). (lu.se)
Formalin-fixed, paraffin-embedded human pancreas stained with CFTR Mouse Monoclonal Antibody (CFTR/1643). (neobiotechnologies.com)
19,000 full-length human proteins using Monospecific Mouse Monoclonal Antibody (CFTR/1643) to CFTR. (neobiotechnologies.com)
8 Studies on a transgenic cftr knockout mouse model suggest that the absence of CFTR does not affect the ability of the kidney to manage fluid and salt imbalance, but that different salt and fluid transport processes are involved. (bmj.com)
It suggests that CFTR potentiators, which have been developed to treat cystic fibrosis, may also be explored as a starting point for the development of a remedy for celiac disease. (eurasiareview.com)
A 2011 study studied behaviour including motor activity and anxiety levels in mice specifically bred to lack the CFTR gene and exhibit symptoms of CF. Researchers expected to find impairments to the endocannabinoid system in such mice compared to healthy controls. (sensiseeds.com)
They demonstrated that once the mutation had been corrected, the function of the CF transmembrane conductor receptor (CFTR) was restored. (bmj.com)
The characterization of the first anti-mouse Muc6 antibody shows an increased expression of the mucin in pancreatic tissue of Cftr-knockout mice. (ox.ac.uk)
In order to further study Muc6 in mice, our aims were to obtain a specific Muc6 antibody, to validate it and to test it in Cftr deficient mice. (ox.ac.uk)
Cftr-/- mice showed a higher expression of Muc6 at both protein and RNA levels compared with their control Cftr+/+ littermates suggesting that as in the human disease, Muc6 may contribute to the formation of materials that block pancreatic acini and ducts in mouse models of cystic fibrosis. (ox.ac.uk)
MATERIALS AND METHODS: In order to evaluate these regulatory elements in vivo we examined these DHS in a human CFTR gene that was introduced on a yeast artificial chromosome (YAC) into transgenic mice. (ox.ac.uk)
The 310 kb human CFTR YAC was shown to restore the pheno-type of CF-null mice and so is likely to contain most of the regulatory elements required for tissue-specific expression of CFTR. (ox.ac.uk)
The DHS in the first intron is present in a more restricted set of tissues in the mice, although its presence does not show complete concordance with CFTR expression. (ox.ac.uk)
Based on the extensive experience of our team in utilizing gene therapy in vivo and gene editing ex vivo and in vivo, the achievment is to acquire a design and construction of the gene correction technology to target the CFTR gene mutation (delTT394) in the humanized CF mouse model. (uni-tuebingen.de)
Cystic fibrosis (CF) is an autosomal recessive condition caused by the mutation of the cystic fibrosis trans-membrane regulator gene (CFTR) on chromosome 7. (who.int)

Naive DeltaF508 -/- and +/- mice showed no pulmonary inflammation and after inhaled Pseudomonas aeruginosa had similar inflammatory responses and bacterial clearance rates. (lookformedical.com)
Mycobacterium abscessus is the etiological agent of severe pulmonary infections in vulnerable patients, such as those with cystic fibrosis (CF), where it represents a relevant cause of morbidity and mortality. (torvergata.it)
The treatment with liposomes of wild-type as well as CF mice, intratracheally infected with M. abscessus, resulted in about a 2-log reduction of pulmonary mycobacterial burden and a significant reduction of macrophages and neutrophils in bronchoalveolar lavage fluid (BALF). (torvergata.it)
IMPORTANCE Mycobacterium abscessus is an opportunistic pathogen intrinsically resistant to many antibiotics, frequently linked to chronic pulmonary infections, and representing a relevant cause of morbidity and mortality, especially in immunocompromised patients, such as those affected by cystic fibrosis. (torvergata.it)
This situation may occur at high altitude or during respiratory diseases, including chronic obstructive pulmonary disease, sleep apnoea and fibrosis, and during failure of ventilation due to neurological diseases. (ersjournals.com)
In this study, we focus on SPL84, an inhaled ASO-based drug, developed for the treatment of the pulmonary disease cystic fibrosis (CF). Pulmonary drug delivery is challenging, due to a variety of biological, physical, chemical, and structural barriers, especially when targeting the cell nucleus. (researcher.life)

Cystic fibrosis (CF) is an autosomal required intravenous fluid therapy and stable, revealed a thriving child with recessive condition caused by the was discharged after correction of his the following serum biochemical re- mutation of the cystic fibrosis trans- biochemical abnormalities. (who.int)

Can Cannabis Help Prevent or Treat Cystic Fibrosis? (sensiseeds.com)

While mouse versions have contributed inside our knowledge of lung advancement fix and regeneration inherent distinctions between your murine and individual airways requires the introduction of new versions using individual airway epithelial cells. (morainetownshipdems.org)
Sodium and epithelial sodium channels participate in the regulation of the capacitation-associated hyperpolarization in mouse sperm. (umassmed.edu)
Mutants, like humans with cystic fibrosis, also exhibit defective epithelial chloride transport. (utsouthwestern.edu)
Electrophysiological properties were tested using short-circuit current experiments in epithelial cells or freshly isolated mouse intestinal tissue. (bvsalud.org)
Although LC activated the cystic fibrosis transmembrane regulator in epithelial cells, its effect was not apparent in colonic epithelium. (bvsalud.org)
The first epithelial cell layer in the development of mouse embryos, the trophectoderm of blastocysts, requires E-cad for proper function. (biomedcentral.com)
Here, we investigated the capacity of N-cad in supporting epithelial integrity in the mammary gland of adult mice. (biomedcentral.com)

Nearly 1000 cystic fibrosis-causing mutations have been described. (wikipedia.org)
Having two copies of this mutation (one inherited from each parent) is by far the most common cause of cystic fibrosis (CF), responsible for nearly two-thirds of mutations worldwide. (wikipedia.org)
Mutations in this gene have been associated with autosomal recessive disorders such as cystic fibrosis and congenital bilateral aplasia of the vas deferens. (utsouthwestern.edu)

Of particular relevance were pancreatic fibrosis, intestinal obstruction, and absence of the vas deferens. (ox.ac.uk)
The rabbit anti-mouse Muc6 polyclonal antibody seems highly specific to the mouse mucin and will be useful to study pancreatic pathology in cystic fibrosis. (ox.ac.uk)
In patients with CF, abnormal pancreatic secretions obstruct the duct system, leading to autodigestion of the acinar cells, fatty replacement, and ultimately, fibrosis. (medscape.com)

This gene encodes the cystic fibrosis transmembrane regulator and a chloride channel that controls the regulation of other transport pathways. (utsouthwestern.edu)

We found that "knockout" mice lacking one of these transport proteins have a high incidence of calcium oxalate urinary stones, the same type that is most common in human patients with kidney stones. (yale.edu)

Non-resolving inflammation is the main mechanism of morbidity and mortality among patients suffering from cystic fibrosis (CF), the most common life-threatening human genetic disease. (frontiersin.org)

Fecal water content, intestinal peristalsis rate, and smooth muscle contraction were measured in neonatal mice infected with SA-11 rotavirus before and after LC treatment or adult mice. (bvsalud.org)
In vivo, LC significantly reduced the fecal water content, intestinal peristalsis rate, and smooth muscle contraction of mice infected with rotavirus. (bvsalud.org)
In addition, the researchers found that VX-770 could protect gluten-sensitive mice from gluten-induced intestinal symptoms. (eurasiareview.com)
0.05) in both CF mouse models, indicating improved intestinal bile salt absorption. (eur.nl)

Cystic fibrosis mice have been generated by gene targeting but show little lung disease without repeated exposure to bacteria. (lookformedical.com)

Src Kinase Is the Connecting Player between Protein Kinase A (PKA) Activation and Hyperpolarization through SLO3 Potassium Channel Regulation in Mouse Sperm. (umassmed.edu)
Gr1 Compact disc11b Compact disc4 Compact disc8 B220 Ter119) Sca-1+ cells from a green fluorescent protein (GFP) expressing transgenic mouse11 into lethally irradiated receiver mice (Shape 1c ?dd). (morainetownshipdems.org)

To block apoptosis, we conditionally deleted one allele of p53 in Ncadk.i. mice and observed a temporal rescue of alveolar morphology and function. (biomedcentral.com)

The suspected diagnosis of cystic fibrosis was confirmed with a sweat chloride level of 120 mmol/L and homo-zygozity for the Delta F 508 gene on genetic studies. (who.int)

Mice were chosen by researchers at Jackson as the best available model for genetic research, and today genetically uniform strains of mice developed at the lab are used in laboratories all over the world. (asu.edu)
Cystic fibrosis is a severe and debilitating genetic disorder that significantly reduces life expectancy and quality of life in sufferers. (sensiseeds.com)
Cystic fibrosis (CF) is a genetic disorder that affects the lungs and various other organs . (sensiseeds.com)
1 It has already been demonstrated that it can be used to repair defective DNA in mice curing them of genetic disorders, 2 and it has been reported that human embryos can be similarly modified. (bmj.com)

Important areas of individual lung advancement fix and regeneration have already been studied by using mouse versions (Morrisey and Hogan 2010 Although these murine versions have provided beneficial insights into lung homeostasis and regeneration you can find intrinsic differences between your individual and mouse airway epithelia (Rock and roll and Hogan 2011 Rock and roll et al. (morainetownshipdems.org)
Efficient killing of inhaled bacteria in DeltaF508 mice: role of airway surface liquid composition. (lookformedical.com)
Components of underlying disease biology were identified by enrolling sites, with cohorts of interstitial fibrosis, immune dysregulation, and airway disease being most commonly reported. (stanfordchildrens.org)
Macrophage Lysosomal Alkalinization Drives Invasive Aspergillosis in a Mouse Cystic Fibrosis Model of Airway Transplantation. (stanfordchildrens.org)

About 1 in 100 people suffer from celiac disease, but the prevalence is about three times higher in patients who also suffer from cystic fibrosis. (eurasiareview.com)
Cystic fibrosis is characterized by the build-up of thick and sticky mucus in the patients' lungs and intestine. (eurasiareview.com)
Lewis S. Smyth A. Survey of acute renal failure in patients with cystic fibrosis in the UK. (cysticfibrosis.online)
Renal involvement was a frequent and devastating complication of amyloidosis in patients with cystic fibrosis. (cysticfibrosis.online)
T Cystic Fibrosis Patients. (researcher.life)
It is the earliest clinical manifestation of cystic fibrosis (CF) and occurs in either a simple or a complicated form in approximately 16-20% of patients who have CF, though it also occurs in patients who do not have CF. (medscape.com)

BK channels may be homomeric or heteromeric and are composed of four seven-transmembrane subunits that form the selectivity pore of the channel. (health-e-nc.org)

These phenotypes possess mainly been mimicked using major tissue from embryonic individual and mouse lungs (Miura and Shiota 2000 Weaver et al. (morainetownshipdems.org)

Suri R. High-frequency audiometry reveals high prevalence of aminoglycoside ototoxicity in children with cystic fibrosis. (cysticfibrosis.online)

Using a radiotracer technique, ASL volume and Cl(-) concentration ([Cl(-)]) were measured in cultured tracheal epithelia from normal and DeltaF508 -/- mice. (lookformedical.com)
There were no differences in ASL [Cl(-)] in DeltaF508 -/- and normal mice (13.8 +/- 2.6 vs. 17.8 +/- 5.6 meq/l). (lookformedical.com)

Furthermore, anandamide has been shown to be present in mother's milk, and there's evidence to suggest that activation of CB1 receptors is crucial to the suckling response in newborn mice. (sensiseeds.com)

Receptor constructs included the N-terminal 20 residues of human rhodopsin and were co-expressed with Galphaolf and the cystic fibrosis transmembrane regulator to allow electrophysiological measurement of receptor responses. (duke.edu)
We used this assay to examine the receptive ranges of all members of the mouse odorant receptor 42 (MOR42) subfamily. (duke.edu)

2002 Hoffmann IM, Rubin BK, Iskandar SS, Schechter MS, Nagaraj SK, Bitzan MM. Acute renal failure in cystic fibrosis: association with inhaled tobramycin therapy. (cysticfibrosis.online)
PubMed ] A report of a 20-year-old patient with cystic fibrosis who developed acute nonoliguric renal failure associated with inhaled tobramycin. (cysticfibrosis.online)

In this study, we demonstrate broad distribution, as well as cell and nucleus penetration of SPL84 in mouse and monkey lungs. (researcher.life)

MI was induced in male C57Bl/6 mice by ligation of the left anterior descending coronary artery. (lu.se)
C57BL/6 mouse splenocytes were stained with CD45R (B220) Monoclonal Antibody, FITC (Product # 11-0452-82) and 0.25 µg of Rat IgG2b kappa Isotype Control, Super Bright 600 (Product # 63-4031-82) (left) or 0.25 µg of CD3 Monoclonal Antibody, Super Bright 600 (right). (thermofisher.com)

Cystic fibrosis (CF) lung transplant recipients (LTRs) exhibit a disproportionately high rate of life-threatening invasive aspergillosis (IA). (stanfordchildrens.org)

106 MSCs from a transgenic GFP-expressing mouse and found a median of only one 1.8% donor-derived osteopoiesis (range 0 = 5) in keeping with. (morainetownshipdems.org)
The DHS at -20.5 kb is present in the chromatin of most tissues of the transgenic mice, supporting its non-tissue-specific nature. (ox.ac.uk)

A complementary approach uses mice with targeted gene disruption to elucidate the physiological roles of ion exchangers and associated proteins under in vivo conditions. (yale.edu)

2001 Cornacchia M, Zenorini A, Perobelli S, Zanolla L, Mastella G, Braggion C. Prevalence of urinary incontinence in women with cystic fibrosis. (cysticfibrosis.online)

Cystic fibrosis (CF) is a hereditary and life-threatening disorder with persistent lung infections. (uni-tuebingen.de)

For example, work with mice lacking anion exchanger Slc26a6, which can function as an oxalate transporter, revealed a phenotype of calcium oxalate kidney stones. (yale.edu)

Examples of such diseases include cystic fibrosis (CF), Duchenne's muscular dystrophy (DMD) and haemoglobinopathies. (bmj.com)

Starvation induces an increase in intracellular calcium and potentiates the progesterone-induced mouse sperm acrosome reaction. (umassmed.edu)

A polyclonal serum named CP4 was isolated from a rabbit immunized by a mouse Muc6 peptide. (ox.ac.uk)

The lab has been pivotal in research with in vitro fertilization, teratomas, gene replacement therapy for birth defects, and more because its researchers have focused from the beginning on developing the mouse as a model organism. (asu.edu)
Acquisition of antifungal resistance influences virulence attributes of C. auris microevolved strains in mouse systemic fungal infection model. (hcemm.eu)
Thus, we provide a new mouse model to investigate the molecular mechanisms of fibrocystic mastopathy and the transition from benign to malignant tumors. (biomedcentral.com)
We recently addressed the question of interchangeability of the two cadherins by generating a mouse model in which N-cad cDNA was introduced into the E-cad locus. (biomedcentral.com)

Lap-Chee Tsui is a geneticist who discovered the cystic fibrosis (CF) gene, and his research team sequenced human chromosome 7. (asu.edu)

Identification of proteins undergoing tyrosine phosphorylation during mouse sperm capacitation. (umassmed.edu)
Biphasic role of calcium in mouse sperm capacitation signaling pathways. (umassmed.edu)

Description: The 17A2 monoclonal antibody reacts with the mouse CD3 complex. (thermofisher.com)

Transient exposure to calcium ionophore enables in vitro fertilization in sterile mouse models. (umassmed.edu)
CF models have been generated in 4 species (mice, rats, ferrets, and pigs) to enhance our understanding of the CF pathogenesis. (ox.ac.uk)
We aimed to determine the effect of AB on fat absorption in two CF mouse models. (eur.nl)

Cdc42 localized in the CatSper signaling complex regulates cAMP-dependent pathways in mouse sperm. (umassmed.edu)

Control of the low voltage-activated calcium channel of mouse sperm by egg ZP3 and by membrane hyperpolarization during capacitation. (umassmed.edu)

Applications Tested: This 17A2 antibody has been tested by flow cytometric analysis of mouse splenocytes. (thermofisher.com)

DISCUSSION: In conclusion, AB treatment does not improve total fat absorption in CF mice but does decrease fecal loss of bile salts and accelerate long-chain fatty acid (LCFA) absorption. (eur.nl)

As the location of the cystic fibrosis gene is now known, it is possible for doctors and specialists to identify in human fetuses the mutation that causes the fatal disease. (asu.edu)
Cystic fibrosis (CF) a monogenic lethal disease and, therefore, ideally suited for the development of gene therapy. (ox.ac.uk)
Glomerular lesions in cystic fibrosis: possible relation to diabetes mellitus, acquired cyanotic heart disease and cirrhosis of the liver. (cysticfibrosis.online)

INTRODUCTION: Improving fat absorption remains a challenge in cystic fibrosis (CF). Antibiotics (AB) treatment has been shown to improve body weight in CF mice. (eur.nl)

In vitro, ivacaftor does not alter LPS-induced CD80 and tumor necrosis factor alpha mRNA increases in BV2 microglial cells, while augmenting mRNA levels of these markers in mouse macrophages and differentiated human THP-1-derived macrophages. (lu.se)
One gene was highly similar to the human beta-defensin-1 (HBD-1) gene and mouse beta-defensin-1 gene at both the nucleic acid and amino acid levels and was termed rat beta-defensin-1 (RBD-1). (lookformedical.com)
While there have so far been no studies into the potential for cannabinoids to treat human infants with CF, research into mice has yielded promising results. (sensiseeds.com)
Five orthologous gel-forming mucins have been cloned in human and mouse. (ox.ac.uk)

Tsui's research also outlined the mechanisms for the development of cystic fibrosis, which were previously unknown. (asu.edu)

Our results demonstrate a possible role for N-cad in the formation of fibrosis and cysts in the mammary gland. (biomedcentral.com)

Co-expression of accessory proteins was required to allow functional surface expression of some mouse odorant receptors. (duke.edu)

McLaren was the first researcher to grow mouse embryos outside of the womb. (asu.edu)
She experimented by culturing mouse eggs and successfully developing them into embryos, leading to advancements with in vitro fertilization. (asu.edu)

Anatomy39

Organisms15

Diseases15

Chemicals and Drugs103

Analytical, Diagnostic and Therapeutic Techniques and Equipment28

Phenomena and Processes55

Disciplines and Occupations2

Technology, Industry, Agriculture1

Information Science3

Health Care2

Conductance18

CFTR18

Pulmonary6

Mutation of the cystic fibrosis1

Treat cystic fibrosis1

Epithelial7

Mutations3

Pancreatic3

Encodes1

Knockout1

Morbidity and morta1

Intestinal4

Lung disease1

Protein2

Conditionally1

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Airway4

Patients6

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20001

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DeltaF5082

Newborn1

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Acute2

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C57Bl2

Exhibit1

Transgenic2

Disruption1

Prevalence1

Disorder1

Anion1

Diseases1

Acrosome reaction1

Rabbit1

Model4

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Sperm Capacitation2

Monoclonal Antibody1

Models3

Regulates1

Membrane1

Cytometric analysis1

Fecal1

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Human4

Mechanisms1

Role1

Proteins1

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