Stefanie Krick
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Assessment of efficacy and tolerability of elexacaftor-tezacaftor-ivacaftor in an observational cohort study of “aged” people with cystic fibrosis Open
Background: Therapeutic advancements utilizing modulators of the cystic fibrosis transmembrane conductance regulator (CFTR) have revolutionized the treatment of people with cystic fibrosis (pwCF). Elexacaftor-Tezacaftor-Ivacaftor (ETI) is …
Elevated glucose increases <i>Staphylococcus aureus</i> antibiotic resistance in a cystic fibrosis airway epithelial cell infection model Open
In a healthy lung, the airway epithelium regulates glucose transport to maintain low glucose concentrations in the airway surface liquid (ASL). However, hyperglycemia and chronic lung diseases, such as cystic fibrosis (CF), can result in i…
Wound repair and immune function in the Pseudomonas infected CF lung: before and after highly effective modulator therapy Open
The leading cause of death for people with cystic fibrosis (pwCF) continues to be due to respiratory-related illnesses. Both wound repair and immune cell responses are dysregulated in the CF airways, creating a cycle of unresolved injury a…
Highly Effective Modulator Therapy: Implications for the Microbial Landscape in Cystic Fibrosis Open
Cystic fibrosis (CF) is an autosomal recessive multisystem disorder caused by mutations in the cystic fibrosis conductance regulator (CFTR) anion channel. In the lungs specifically, CFTR mutations lead to changes in mucus viscosity and def…
View article: Hyperphosphatemia Contributes to Skeletal Muscle Atrophy in Mice
Hyperphosphatemia Contributes to Skeletal Muscle Atrophy in Mice Open
Chronic kidney disease (CKD) is associated with various pathologic changes, including elevations in serum phosphate levels (hyperphosphatemia), vascular calcification, and skeletal muscle atrophy. Elevated phosphate can damage vascular smo…
View article: FGF receptors mediate cellular senescence in the cystic fibrosis airway epithelium
FGF receptors mediate cellular senescence in the cystic fibrosis airway epithelium Open
The number of adults living with cystic fibrosis (CF) has already increased significantly because of drastic improvements in life expectancy attributable to advances in treatment, including the development of highly effective modulator the…
View article: O-GlcNAc transferase regulates collagen deposition and fibrosis resolution in idiopathic pulmonary fibrosis
O-GlcNAc transferase regulates collagen deposition and fibrosis resolution in idiopathic pulmonary fibrosis Open
Background Idiopathic pulmonary fibrosis (IPF) is a chronic pulmonary disease that is characterized by an excessive accumulation of extracellular matrix (ECM) proteins (e.g. collagens) in the parenchyma, which ultimately leads to respirato…
407 The OGT/O-GlcNAc Axis Regulates Fibrosis Resolution in Idiopathic Pulmonary Fibrosis Open
OBJECTIVES/GOALS: Idiopathic pulmonary fibrosis (IPF) is a chronic lung disease characterized by dysregulated collagen accumulation in the lung parenchyma. Our goal is to investigate the role of O-linked N-Acetylglucosamine (O-GlcNAc) tran…
O‐GlcNAc regulates anti‐fibrotic genes in lung fibroblasts through EZH2 Open
Epigenetic modifications are involved in fibrotic diseases, such as idiopathic pulmonary fibrosis (IPF), and contribute to the silencing of anti‐fibrotic genes. H3K27me3, a key repressive histone mark, is catalysed by the methyltransferase…
What the future holds: cystic fibrosis and aging Open
Cystic fibrosis (CF) is one of the most common genetic diseases with around 70,000 affected patients worldwide. CF is a multisystem disease caused by a mutation in the CF transmembrane conductance regulator gene, which has led to a signifi…
View article: Systematic Review of the Methodological Quality and Outcome Measures Utilized in Exercise Interventions for Individuals with Cystic Fibrosis Methodological Quality and Outcome Measures Utilized in CF Exercise Studies
Systematic Review of the Methodological Quality and Outcome Measures Utilized in Exercise Interventions for Individuals with Cystic Fibrosis Methodological Quality and Outcome Measures Utilized in CF Exercise Studies Open
Background: The aims of this study were to evaluate the methodological quality of interventional exercise studies in individuals with cystic fibrosis (CF) and link the reported outcome measures of these studies to the International Classif…
View article: An ex vivo rat trachea model reveals abnormal airway physiology and a gland secretion defect in cystic fibrosis
An ex vivo rat trachea model reveals abnormal airway physiology and a gland secretion defect in cystic fibrosis Open
Cystic fibrosis (CF) is a genetic disease hallmarked by aberrant ion transport that results in delayed mucus clearance, chronic infection, and progressive lung function decline. Several animal models have been developed to study the airway…
View article: Fibroblast Growth Factor 23 Signaling Does Not Increase Inflammation from Pseudomonas aeruginosa Infection in the Cystic Fibrosis Bronchial Epithelium
Fibroblast Growth Factor 23 Signaling Does Not Increase Inflammation from Pseudomonas aeruginosa Infection in the Cystic Fibrosis Bronchial Epithelium Open
Background and Objectives: Chronic inflammation due to Pseudomonas aeruginosa (PA) infection in people with cystic fibrosis (CF) remains a concerning issue in the wake of modulator therapy initiation. Given the perpetuating cycle of coloni…
View article: A novel in vitro model to study prolonged Pseudomonas aeruginosa infection in the cystic fibrosis bronchial epithelium
A novel in vitro model to study prolonged Pseudomonas aeruginosa infection in the cystic fibrosis bronchial epithelium Open
Pseudomonas aeruginosa (PA) is known to chronically infect airways of people with cystic fibrosis (CF) by early adulthood. PA infections can lead to increased airway inflammation and lung tissue damage, ultimately contributing to decreased…
Fibroblast Growth Factor Signaling in Development and Disease Open
Fibroblast growth factors (FGFs) and their cognate receptors (FGFRs) are important biological molecules with a wide array of pleiotropic functions [...]
Phosphate induces inflammation and exacerbates injury from cigarette smoke in the bronchial epithelium Open
An elevation in serum phosphate—also called hyperphosphatemia—is associated with reduced kidney function in chronic kidney disease (CKD). Reports show CKD patients are more likely to develop lung disease and have poorer kidney function tha…