Frauke Stanke
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View article: NLRP3 regulates epithelial barrier integrity and protects from airway hyperresponsiveness in experimental allergic asthma
NLRP3 regulates epithelial barrier integrity and protects from airway hyperresponsiveness in experimental allergic asthma Open
Background The inflammasome NLRP3 (NOD-like receptor family pyrin domain containing 3) is critical for epithelial barrier integrity. Allergic asthma is characterized by airway inflammation, airway hyperresponsiveness (AHR), and mucus hyper…
View article: Human induced pluripotent stem cells for in vitro modeling of impaired mucociliary clearance in cystic fibrosis lung disease
Human induced pluripotent stem cells for in vitro modeling of impaired mucociliary clearance in cystic fibrosis lung disease Open
Severely impaired mucociliary airway function is the primary pathomechanism in Cystic Fibrosis (CF) lung disease. Despite significant advances in CF therapy, there is still a critical need for alternative, individualized treatment options,…
View article: Genotype and transcript processing of the tumour necrosis factor receptor TNFRSF1A in epithelial cells: implications for survival in cystic fibrosis
Genotype and transcript processing of the tumour necrosis factor receptor TNFRSF1A in epithelial cells: implications for survival in cystic fibrosis Open
Supported by the German Ministry for Education and Research (BMBF) (82DZL009B1 to MAM and 82DZL002A1, to GH, BT, AMD, FS) and the Mukoviszidose Institut gGmbH (MI-2002, to LN, AMD, FS).
View article: Analysis of CFTR mRNA and Protein in Peripheral Blood Mononuclear Cells via Quantitative Real-Time PCR and Western Blot
Analysis of CFTR mRNA and Protein in Peripheral Blood Mononuclear Cells via Quantitative Real-Time PCR and Western Blot Open
The Cystic Fibrosis Conductance Transmembrane Regulator gene encodes for the CFTR ion channel, which is responsible for the transport of chloride and bicarbonate across the plasma membrane. Mutations in the gene result in impaired ion tran…
View article: A Fast Scoring of Human Primary Respiratory Epithelia Grown at Air–Liquid Interface (ALI) to Assess Epithelial Morphology in Research and Personalized Medicine Settings
A Fast Scoring of Human Primary Respiratory Epithelia Grown at Air–Liquid Interface (ALI) to Assess Epithelial Morphology in Research and Personalized Medicine Settings Open
Background: In recent years, increasingly complex ALI protocols involving specialized, albeit laboratory-specific media have been established, while at the same time, many studies compile the data of only a few ALI donors in spite of site-…
View article: Editorial: Acute phase proteins as biomarkers and therapeutics in acute and chronic inflammatory conditions
Editorial: Acute phase proteins as biomarkers and therapeutics in acute and chronic inflammatory conditions Open
EDITORIAL article Front. Pharmacol., 02 February 2023Sec. Inflammation Pharmacology Volume 14 - 2023 | https://doi.org/10.3389/fphar.2023.1145990
View article: Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy
Changes in cystic fibrosis transmembrane conductance regulator protein expression prior to and during elexacaftor-tezacaftor-ivacaftor therapy Open
Background: Defects in expression, maturation or function of the epithelial membrane glycoprotein CFTR are causative for the progressive disease cystic fibrosis. Recently, molecular therapeutics that improve CFTR maturation and functional …
View article: Complementary Dual Approach for In Silico Target Identification of Potential Pharmaceutical Compounds in Cystic Fibrosis
Complementary Dual Approach for In Silico Target Identification of Potential Pharmaceutical Compounds in Cystic Fibrosis Open
Cystic fibrosis is a genetic disease caused by mutation of the CFTR gene, which encodes a chloride and bicarbonate transporter in epithelial cells. Due to the vast range of geno- and phenotypes, it is difficult to find causative treatments…
View article: Generation of two TMEM16A knockout iPSC clones each from a healthy human iPSC line, from a Cystic Fibrosis patient specific line with p.Phe508del mutation and from the gene corrected iPSC line
Generation of two TMEM16A knockout iPSC clones each from a healthy human iPSC line, from a Cystic Fibrosis patient specific line with p.Phe508del mutation and from the gene corrected iPSC line Open
The Transmembrane member 16A (TMEM16A), also known as anoctamin-1 (ANO1), is a calcium-activated chloride channel present in the airway epithelium. It is known to be involved in the apical chloride secretion indicating that TMEM16A could b…
View article: Integrating Text Mining into the Curation of Disease Maps
Integrating Text Mining into the Curation of Disease Maps Open
An adequate visualization form is required to gain an overview and ultimately understand the complex and diverse biological mechanisms of diseases. Recently, disease maps have been introduced for this purpose. A disease map is defined as a…
View article: Analysis of CF patient survival confirms STAT3 as a CF-modifying gene with changing impact over time
Analysis of CF patient survival confirms STAT3 as a CF-modifying gene with changing impact over time Open
Introduction and aim The signal transducer and activator of transcription 3 (STAT3) has been identified as one of the cystic fibrosis (CF) modifying genes. In this study, we aimed to assess the association between STAT3 genotype and CF pat…
View article: Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells
Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells Open
Cystic fibrosis (CF) is a monogenic disease, characterized by massive chronic lung inflammation. The observed variability in clinical phenotypes in monozygotic CF twins is likely associated with the extent of inflammation. This study sough…
View article: Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR
Comprehensive Analysis of Chemical Structures That Have Been Tested as CFTR Activating Substances in a Publicly Available Database CandActCFTR Open
Background: Cystic fibrosis (CF) is a genetic disease caused by mutations in CFTR , which encodes a chloride and bicarbonate transporter expressed in exocrine epithelia throughout the body. Recently, some therapeutics became available that…
View article: Consistent Assignment of Risk and Benign Allele at rs2303153 in the CF Modifier Gene SCNN1B in Three Independent F508del-CFTR Homozygous Patient Populations
Consistent Assignment of Risk and Benign Allele at rs2303153 in the CF Modifier Gene SCNN1B in Three Independent F508del-CFTR Homozygous Patient Populations Open
CFTR encodes for a chloride and bicarbonate channel expressed at the apical membrane of polarized epithelial cells. Transepithelial sodium transport mediated by the amiloride-sensitive sodium channel ENaC is thought to contribute to the ma…
View article: Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in<i>PKP3</i>in lung epithelial cells
Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in<i>PKP3</i>in lung epithelial cells Open
Cystic fibrosis (CF) is a monogenic disease, characterized by massive chronic lung inflammation. The observed variability in clinical phenotypes in monozygotic CF twins is likely associated with the extent of inflammation. This study sough…
View article: CFTR Lifecycle Map—A Systems Medicine Model of CFTR Maturation to Predict Possible Active Compound Combinations
CFTR Lifecycle Map—A Systems Medicine Model of CFTR Maturation to Predict Possible Active Compound Combinations Open
Different causative therapeutics for CF patients have been developed. There are still no mutation-specific therapeutics for some patients, especially those with rare CFTR mutations. For this purpose, high-throughput screens have been perfo…
View article: Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface
Effect of Alpha-1 Antitrypsin on CFTR Levels in Primary Human Airway Epithelial Cells Grown at the Air-Liquid-Interface Open
The cystic fibrosis transmembrane conductance regulator (CFTR) gene is influenced by the fundamental cellular processes like epithelial differentiation/polarization, regeneration and epithelial–mesenchymal transition. Defects in CFTR prote…
View article: VJ Segment Usage of TCR-Beta Repertoire in Monozygotic Cystic Fibrosis Twins
VJ Segment Usage of TCR-Beta Repertoire in Monozygotic Cystic Fibrosis Twins Open
Sixteen monozygotic cystic fibrosis (CF) twin pairs of whom 14 pairs were homozygous for the most common p.Phe508del CFTR mutation were selected from the European Cystic Fibrosis Twin and Sibling Study Cohort. The monozygotic twins were ex…
View article: Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells
Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells Open
Cystic fibrosis (CF) is a monogenic disease, characterized by massive chronic lung inflammation. The observed variability in clinical phenotypes in monozygotic CF twins is likely associated with the extent of inflammation. This study sough…
View article: Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells
Disease-related blood-based differential methylation in cystic fibrosis and its representation in lung cancer revealed a regulatory locus in <i>PKP3</i> in lung epithelial cells Open
Cystic fibrosis (CF) is a monogenic disease, characterized by massive chronic lung inflammation. The observed variability in clinical phenotypes in monozygotic CF twins is likely associated with the extent of inflammation. This study sough…
View article: Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive <i>CFTR</i> genetics and sweat test
Intestinal current measurement and nasal potential difference to make a diagnosis of cases with inconclusive <i>CFTR</i> genetics and sweat test Open
Background Nasal potential difference (NPD) and intestinal current measurements (ICM) are cystic fibrosis transmembrane conductance regulator (CFTR) biomarkers recommended to make a diagnosis in individuals with inconclusive sweat test and…
View article: Functional analysis of the p.[Arg74Trp;Val201Met;Asp1270Asn]/p.Phe508del <i>CFTR</i> mutation genotype in human native colon
Functional analysis of the p.[Arg74Trp;Val201Met;Asp1270Asn]/p.Phe508del <i>CFTR</i> mutation genotype in human native colon Open
Background The impact of complex alleles on CFTR processing and function has yet not been investigated in native human tissue. Methods Intestinal current measurements (ICM) followed by CFTR immunoblot were performed on rectal biopsies take…
View article: Nasal potential difference of carriers of the W493R ENaC variant with non-cystic fibrosis bronchiectasis
Nasal potential difference of carriers of the W493R ENaC variant with non-cystic fibrosis bronchiectasis Open
Common underlying conditions leading to bronchiectasis are infection, immune deficiency or cystic fibrosis (CF) [1]. Transgenic mice that overexpress the amiloride-sensitive epithelial sodium channel ENaC mimic the typical features of CF l…