A5073951705- Cystic Fibrosis Research Advances
- Neonatal Respiratory Health Research
- Esophageal and GI Pathology
- Drug Transport and Resistance Mechanisms
- Tracheal and airway disorders
- Asthma and respiratory diseases
- Eosinophilic Esophagitis
- Mitochondrial Function and Pathology
- Child Nutrition and Feeding Issues
- Pharmaceutical studies and practices
- Inhalation and Respiratory Drug Delivery
- Ion Transport and Channel Regulation
- Helicobacter pylori-related gastroenterology studies
- Genetic and Kidney Cyst Diseases
- Microtubule and mitosis dynamics
- Erythrocyte Function and Pathophysiology
- Cellular Mechanics and Interactions
- Protein Kinase Regulation and GTPase Signaling
- Autophagy in Disease and Therapy
- Cellular transport and secretion
- Congenital Ear and Nasal Anomalies
- Wnt/β-catenin signaling in development and cancer
- Epigenetics and DNA Methylation
- Advanced biosensing and bioanalysis techniques
- Molecular Communication and Nanonetworks
Cedars-Sinai Medical Center
2023-2024
University of Alabama at Birmingham
2023
Emory University
2023
Sinai Hospital
2023
Cystic Fibrosis Foundation
2023
Children's Healthcare of Atlanta
2023
Children's Center
2023
Cincinnati Children's Hospital Medical Center
2014-2022
University of Cincinnati Medical Center
2021
University of Cincinnati
2021
Transmitochondrial cybrids and multiple OMICs approaches were used to understand mitochondrial reprogramming mitochondria-regulated cancer pathways in triple-negative breast (TNBC). Analysis of established (BC) cell lines showed that metastatic TNBC maintains high levels ATP through fatty acid β oxidation (FAO) activates Src oncoprotein autophosphorylation at Y419. Manipulation FAO including the knocking down carnitine palmitoyltransferase-1A (CPT1) 2 (CPT2), rate-limiting proteins FAO,...
Abstract Cystic fibrosis (CF) is a genetic disorder caused by defective CF Transmembrane Conductance Regulator (CFTR) function. Insulin producing pancreatic islets are located in close proximity to the duct and there possibility of impaired cell-cell signaling between ductal epithelial cells (PDECs) islet as causative CF. To study this possibility, we present an vitro co-culturing system, pancreas-on-a-chip. Furthermore, efficient method micro dissect patient-derived human ducts from remnant...
Pulmonary function is dependent upon the precise regulation of alveolar surfactant. Alterations in pulmonary surfactant concentrations or impair ventilation and cause tissue injury. Identification molecular pathways that sense regulate endogenous concentrations, coupled with ability to pharmacologically modulate them both positively negatively, would be a major therapeutic advance for patients acute chronic lung diseases caused by disruption homeostasis. The orphan adhesion GPCR GPR116 (also...
It has long been known that cyclic nucleotides and nucleotide-dependent signaling molecules control cell migration. However, the concept it is not just absence or presence of nucleotides, but a highly coordinated balance between these regulates migration, new revolutionary. In this study, we used multidrug resistance protein 4 (MRP4)-expressing lines MRP4 knock-out mice as model systems wound healing assays experimental system to explore unique emerging concept. MRP4, member large family ATP...
Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in CFTR, plasma-membrane-localized anion channel. The most common mutation deletion of phenylalanine at residue 508 (ΔF508), causes misfolding CFTR resulting little or no protein the plasma membrane. corrector VX-809 shows promise for treating CF patients homozygous ΔF508. Here, we demonstrate significance protein–protein interactions enhancing stability ΔF508 mutant channel We determined that prolongs Using...
Cystic fibrosis (CF) is a lethal genetic disease caused by the loss or dysfunction of CF transmembrane conductance regulator (CFTR) channel. F508del most prevalent mutation CFTR gene and encodes protein defective in folding processing. VX-809 has been reported to facilitate trafficking F508del-CFTR augment its channel function. The mechanism action poorly understood. In this study, we sought answer fundamental question underlying VX-809: does it bind directly order exert action? We...
<h2>Summary</h2> Cystic fibrosis (CF) is a genetic disease caused by mutation in the cystic transmembrane conductance regulator (CFTR) gene. Despite reports of CFTR expression on endothelial cells, pulmonary vascular perturbations, and perfusion deficits CF patients, mechanism remains unclear. Here, our pilot study 40 patients reveals loss small blood vessels with severe lung disease. Using vessel-on-a-chip model, we establish shear-stress-dependent barrier failure involving TRPV4,...
Cystic fibrosis (CF) is a genetic disorder in which epithelium-generated fluid flow from the lung, intestine, and pancreas impaired due to mutations disrupting CF transmembrane conductance regulator (CFTR) channel function. manifestations of lung are present vast majority patients, 15% infants born with obstructed gut or meconium ileus. However, constipation significantly underreported outcome disease, affecting 47% management becomes critical wake increasing life span patients. In this...
Abstract The molecular mechanism of Endoplasmic Reticulum-associated degradation (ERAD) Cystic fibrosis transmembrane-conductance regulator (CFTR) is largely unknown. Particularly, it unknown what ER luminal factor(s) are involved in ERAD. Herein, we used ProtoArray to identify an co-chaperone, DNAJB9, which can directly interact with CFTR. For both WT- and ΔF508 (deletion phenylalanine at position 508, the most common CF-causing mutant)-CFTR, knockdown DNAJB9 by siRNA increased their...
Motile cilia are hairlike structures that line the respiratory and reproductive tracts middle ear generate fluid flow in these organs via synchronized beating. Cilium growth is a highly regulated process assumed to be important for generation. Recently, Kif19a, kinesin residing at tip, was identified essential ciliary length control through its microtubule depolymerization function. However, there lack of information on nature proteins integrated signaling mechanism regulating motile cilia....
Cystic fibrosis (CF) is a life-shortening disease caused by the mutations that generate nonfunctional CF transmembrane conductance regulator (CFTR) protein. A rare serine-to-tyrosine (S1045Y) CFTR mutation was earlier reported to result in CF-associated fatality. We identified an African-American patient with S1045Y CFTR, as well stop-codon mutation, who has mild phenotype. The underlying mechanism of S1045Y-CFTR not been elucidated. In this study, we determined exhibits twofold attenuated...
Autophagy, a cellular stress response to starvation and bacterial infection, is executed by double-membrane-bound organelles called autophagosomes. Autophagosomes transfer cytosolic material acidified lysosomes for degradation following soluble N-ethylmaleimide-sensitive factor attachment receptor (SNARE)-dependent fusion processes. Many of the autophagy-related disorders stem from defective end-step proteolysis inside lysosomes. The role epithelial cystic fibrosis (CF) transmembrane...