Susan E. Birket
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View article: Pseudomonas aeruginosa increases viscoelasticity and decreases transportability of artificial mucus
Pseudomonas aeruginosa increases viscoelasticity and decreases transportability of artificial mucus Open
View article: Targeting the MEK1/2 pathway to combat <i>Staphylococcus aureus</i> infection and inflammation in cystic fibrosis
Targeting the MEK1/2 pathway to combat <i>Staphylococcus aureus</i> infection and inflammation in cystic fibrosis Open
Staphylococcus aureus infections remain an ongoing challenge for people with cystic fibrosis (PwCF), with the increased global prevalence of multidrug-resistant strains requiring new therapeutic approaches. Our previous studies demonstrate…
View article: <i>Aspergillus fumigatus</i>is influenced by mucus accumulation, airway inflammation, and CFTR function
<i>Aspergillus fumigatus</i>is influenced by mucus accumulation, airway inflammation, and CFTR function Open
Background Aspergillus fumigatus (Af) is an inhaled mold found in people with CF (PwCF) that results in significant airway inflammation. Studies have shown allergic (Th2) inflammatory responses to Af, little change in allergic bronchopulmo…
View article: Spontaneous lung colonization in the cystic fibrosis rat model is linked to gastrointestinal obstruction
Spontaneous lung colonization in the cystic fibrosis rat model is linked to gastrointestinal obstruction Open
Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene, resulting in CFTR protein dysfunction. CFTR dysfunction has multi-organ consequences, leading to dehydr…
View article: Ivacaftor ameliorates mucus burden, bacterial load, and inflammation in acute but not chronic P. aeruginosa infection in hG551D rats
Ivacaftor ameliorates mucus burden, bacterial load, and inflammation in acute but not chronic P. aeruginosa infection in hG551D rats Open
The data indicate that the hG551D rats have better outcomes during infection when treated with ivacaftor compared to the vehicle group. Rats have increased weight gain, increased CFTR protein function, and decreased mucus accumulation, des…
View article: Evaluating the effects of ivacaftor exposure on <i>Staphylococcus aureus</i> small colony variant development and antibiotic tolerance
Evaluating the effects of ivacaftor exposure on <i>Staphylococcus aureus</i> small colony variant development and antibiotic tolerance Open
Background Ivacaftor exhibits anti-staphylococcal properties but does not clear Staphylococcus aureus from the lungs of people with cystic fibrosis (pwCF). We assessed whether exposure to therapeutic concentrations of ivacaftor could allow…
View article: Normalization of Muc5b ameliorates airway mucus plugging during persistent <i>Pseudomonas aeruginosa</i> infection in the CFTR<sup>–/–</sup> rat
Normalization of Muc5b ameliorates airway mucus plugging during persistent <i>Pseudomonas aeruginosa</i> infection in the CFTR<sup>–/–</sup> rat Open
Although highly effective modulator therapies for cystic fibrosis (CF) have improved mucus-related outcomes of disease for people with CF, eradication of Pseudomonas aeruginosa (PA) infection has not been achieved in this population. In ad…
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: The big impact of small Airway pH
The big impact of small Airway pH Open
Cystic fibrosis (CF) lung disease, caused by abnormal ion transport because of deficient CFTR (cystic fibrosis transmembrane conductance regulator) function, is characterized by a dehydrated, hyperconcentrated mucus layer, leading to persi…
View article: Metachrony drives effective mucociliary transport via a calcium-dependent mechanism
Metachrony drives effective mucociliary transport via a calcium-dependent mechanism Open
We developed a novel imaging-based analysis to detect coordination of ciliary motion and optimal coordination, a process called metachrony. We found that metachrony is key to the optimization of ciliary-mediated mucus transport in both fer…
View article: 48 Spontaneous infection in the cystic fibrosis rat model is linked to gastrointestinal obstruction
48 Spontaneous infection in the cystic fibrosis rat model is linked to gastrointestinal obstruction Open
View article: 47 Novel detection and infection modeling of Aspergillus fumigatus in pyocyanin-stimulated primary airway epithelium and mucus models
47 Novel detection and infection modeling of Aspergillus fumigatus in pyocyanin-stimulated primary airway epithelium and mucus models Open
View article: 212 Ivacaftor ameliorates acute but not chronic Pseudomonas aeruginosa infection in cystic fibrosis rats
212 Ivacaftor ameliorates acute but not chronic Pseudomonas aeruginosa infection in cystic fibrosis rats Open
View article: Transgenic ferret models define pulmonary ionocyte diversity and function
Transgenic ferret models define pulmonary ionocyte diversity and function Open
View article: SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease
SNSP113 (PAAG) improves mucociliary transport and lung pathology in the Scnn1b-Tg murine model of CF lung disease Open
View article: Repurposing sunscreen as an antibiotic: zinc-activated avobenzone inhibits methicillin-resistant <i>Staphylococcus aureus</i>
Repurposing sunscreen as an antibiotic: zinc-activated avobenzone inhibits methicillin-resistant <i>Staphylococcus aureus</i> Open
Methicillin-resistant Staphylococcus aureus (MRSA) is a major healthcare concern with associated healthcare costs reaching over ${\$}$1 billion in a single year in the USA. Antibiotic resistance in S. aureus is now observed against last li…
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…
View article: Engineered tRNAs suppress nonsense mutations in cells and in vivo
Engineered tRNAs suppress nonsense mutations in cells and in vivo Open
View article: ATP12A: Connecting Mucus and Fibrosis in Idiopathic Pulmonary Fibrosis
ATP12A: Connecting Mucus and Fibrosis in Idiopathic Pulmonary Fibrosis Open
"ATP12A: Connecting Mucus and Fibrosis in IPF." American Journal of Respiratory Cell and Molecular Biology, 0(ja), pp.
View article: Acute Infection with a Tobramycin-Induced Small Colony Variant of Staphylococcus aureus Causes Increased Inflammation in the Cystic Fibrosis Rat Lung
Acute Infection with a Tobramycin-Induced Small Colony Variant of Staphylococcus aureus Causes Increased Inflammation in the Cystic Fibrosis Rat Lung Open
Cystic fibrosis (CF) disease is characterized by lifelong infections with pathogens such as Staphylococcus aureus , leading to eventual respiratory failure. Small colony variants (SCVs) of S. aureus have been linked to worse clinical outco…
View article: A step forward for an intermediate cystic fibrosis population
A step forward for an intermediate cystic fibrosis population Open
Thao Nguyen-Khoa and colleagues investigated the benefit of using beta-adrenergic (β-adr) sweat secretion (SST) measured by evaporimetry to determine the CFTR functional status, an important report that presents a useful novel method.
View article: Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis
Muc5b Contributes to Mucus Abnormality in Rat Models of Cystic Fibrosis Open
Cystic fibrosis (CF) airway disease is characterized by excessive and accumulative mucus in the airways. Mucociliary clearance becomes defective as mucus secretions become hyperconcentrated and viscosity increases. The CFTR-knockout (KO) r…
View article: Static mucus impairs bacterial clearance and allows chronic infection with<i>Pseudomonas aeruginosa</i>in the cystic fibrosis rat
Static mucus impairs bacterial clearance and allows chronic infection with<i>Pseudomonas aeruginosa</i>in the cystic fibrosis rat Open
Cystic fibrosis airway disease is characterised by chronic Pseudomonas aeruginosa infection. Successful eradication strategies have been hampered by a poor understanding of the mechanisms underlying conversion to chronicity. The cystic fib…
View article: Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model
Poly (acetyl, arginyl) glucosamine disrupts Pseudomonas aeruginosa biofilms and enhances bacterial clearance in a rat lung infection model Open
Pseudomonas aeruginosa is a common opportunistic pathogen that can cause chronic infections in multiple disease states, including respiratory infections in patients with cystic fibrosis (CF) and non-CF bronchiectasis. Like many opportunist…
View article: Inhaled gene therapy of preclinical muco-obstructive lung diseases by nanoparticles capable of breaching the airway mucus barrier
Inhaled gene therapy of preclinical muco-obstructive lung diseases by nanoparticles capable of breaching the airway mucus barrier Open
Introduction Inhaled gene therapy of muco-obstructive lung diseases requires a strategy to achieve therapeutically relevant gene transfer to airway epithelium covered by particularly dehydrated and condensed mucus gel layer. Here, we intro…
View article: 401: Muc5b knockdown alters chronic infection outcomes in CFTR-KO rats
401: Muc5b knockdown alters chronic infection outcomes in CFTR-KO rats Open
View article: 566: Accelerated aging pathways are activated in cystic fibrosis airway disease
566: Accelerated aging pathways are activated in cystic fibrosis airway disease Open
View article: 507: Establishing a mutant for modeling Staphylococcus aureus small colony–variant airway infection in cystic fibrosis
507: Establishing a mutant for modeling Staphylococcus aureus small colony–variant airway infection in cystic fibrosis Open
View article: 384: Advances of magnetomotive micro-optical coherence tomography for mucus microrheology
384: Advances of magnetomotive micro-optical coherence tomography for mucus microrheology Open
View article: Ivacaftor partially corrects airway inflammation in a humanized G551D rat
Ivacaftor partially corrects airway inflammation in a humanized G551D rat Open
Animal models have been highly informative for understanding the pathogenesis and progression of cystic fibrosis (CF) lung disease. In particular, the CF rat models recently developed have addressed mechanistic causes of the airway mucus d…