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View article: Compound Heterozygous <i>MRPS14</i> Variants Associated With Leigh Syndrome
Compound Heterozygous <i>MRPS14</i> Variants Associated With Leigh Syndrome Open
MRPS14 (uS14m) is a nuclear‐encoded ribosomal protein important for mitochondria‐specific translation. To date, only a single individual with a recessive MRPS14 ‐related disorder (also known as COXPD38) has been reported. We report an addi…
View article: IMPACT OF LOW PROTEIN DIETS ON GUT HEALTH
IMPACT OF LOW PROTEIN DIETS ON GUT HEALTH Open
Introduction Studies on the gut-brain axis report that changes in the microbiome contribute to age-related diseases. One of the most significant factors affecting microbiome composition is diet. Prior work from our lab has demonstrated tha…
View article: DIETARY PROTEIN RESTRICTION IN AGED MALE MICE REDUCES MARKERS OF SASP IN ADIPOSE TISSUE
DIETARY PROTEIN RESTRICTION IN AGED MALE MICE REDUCES MARKERS OF SASP IN ADIPOSE TISSUE Open
Aging is associated with cellular senescence and metabolic impairment of adipose tissue. Moreover, the detriments of aging are further exacerbated by obesity on metabolic function in adipose tissue. Our lab and others have shown that dieta…
View article: THE IMPACT OF DIETARY PROTEIN RESTRICTION ON WHITE ADIPOSE TISSUE FUNCTION DURING AGING
THE IMPACT OF DIETARY PROTEIN RESTRICTION ON WHITE ADIPOSE TISSUE FUNCTION DURING AGING Open
White adipose tissue (WAT) regulates metabolic health, lipid storage and energy mobilization, yet these functions decline during aging and aging with obesity. Studies show that interventions such as dietary protein restriction (DPR) can im…
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…
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…
View article: Phosphate induces inflammation and exacerbates injury from cigarette smoke in the bronchial epithelium
Phosphate induces inflammation and exacerbates injury from cigarette smoke in the bronchial epithelium Open
View article: 429 Elevated Phosphate Levels Induce Lung Inflammation and Exacerbate Pulmonary Fibrosis
429 Elevated Phosphate Levels Induce Lung Inflammation and Exacerbate Pulmonary Fibrosis Open
OBJECTIVES/GOALS: Using a cell culture model, we will determine the effects of phosphate on primary lung cell cultures and use our results to delineate a pathway through which these changes are carried out. Using animal models, we will det…
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: ST6GAL1 and α2-6 Sialylation Regulates IL-6 Expression and Secretion in Chronic Obstructive Pulmonary Disease
ST6GAL1 and α2-6 Sialylation Regulates IL-6 Expression and Secretion in Chronic Obstructive Pulmonary Disease Open
Chronic obstructive pulmonary disease (COPD) is a systemic disease strongly associated with cigarette smoking, airway inflammation, and acute disease exacerbations. Changes in terminal sialylation and fucosylation of asparagine (N)-linked …
View article: Cystic Fibrosis Lung Disease in the Aging Population
Cystic Fibrosis Lung Disease in the Aging Population Open
The demographics of the population with cystic fibrosis (CF) is continuously changing, with nowadays adults outnumbering children and a median predicted survival of over 40 years. This leads to the challenge of treating an aging CF populat…
View article: Targeting Aging Pathways in Chronic Obstructive Pulmonary Disease
Targeting Aging Pathways in Chronic Obstructive Pulmonary Disease Open
Chronic obstructive pulmonary disease (COPD) has become a global epidemic and is the third leading cause of death worldwide. COPD is characterized by chronic airway inflammation, loss of alveolar-capillary units, and progressive decline in…
View article: Fibroblast Growth Factor Receptor 4 Deficiency Mediates Airway Inflammation in the Adult Healthy Lung?
Fibroblast Growth Factor Receptor 4 Deficiency Mediates Airway Inflammation in the Adult Healthy Lung? Open
Fibroblast growth factor receptor (FGFR) 4 has been shown to mediate pro-inflammatory signaling in the liver and airway epithelium in chronic obstructive pulmonary disease. In past reports, FGFR4 knockout (Fgfr4 -/- )<…
View article: Fibroblast Growth Factor Signaling as a Mediator of Inflammation and Vascular Remodeling in Pulmonary Diseases
Fibroblast Growth Factor Signaling as a Mediator of Inflammation and Vascular Remodeling in Pulmonary Diseases Open
View article: The Effects of the Anti-aging Protein Klotho on Mucociliary Clearance
The Effects of the Anti-aging Protein Klotho on Mucociliary Clearance Open
α-klotho (KL) is an anti-aging protein and has been shown to exert anti-inflammatory and anti-oxidative effects in the lung and pulmonary diseases such as chronic obstructive pulmonary disease (COPD) and cystic fibrosis. The current study …