A5090229639- Congenital Diaphragmatic Hernia Studies
- Neonatal Respiratory Health Research
- Glycosylation and Glycoproteins Research
- Congenital Anomalies and Fetal Surgery
- Congenital Heart Disease Studies
- Tracheal and airway disorders
- Galectins and Cancer Biology
- Infant Development and Preterm Care
- Mechanical Circulatory Support Devices
- Urological Disorders and Treatments
- TGF-β signaling in diseases
- Esophageal and GI Pathology
- Hernia repair and management
- Proteoglycans and glycosaminoglycans research
- Hematopoietic Stem Cell Transplantation
- Neonatal and fetal brain pathology
- Ubiquitin and proteasome pathways
- Congenital gastrointestinal and neural anomalies
- Mesenchymal stem cell research
- Teratomas and Epidermoid Cysts
- Pleural and Pulmonary Diseases
- Intestinal Malrotation and Obstruction Disorders
- Cancer, Hypoxia, and Metabolism
- Ultrasound in Clinical Applications
- Polyomavirus and related diseases
Children's Hospital of Philadelphia
2015-2024
University of Pennsylvania
2012-2024
Fetal Medicine Foundation
2023
University of Toronto
2006-2020
Hospital for Sick Children
2020
Canada Research Chairs
2020
Philadelphia University
2014
University of Technology Sydney
2012
Children's Center
2012
Mount Sinai Hospital
2002-2007
The Golgi enzyme beta1,6 N-acetylglucosaminyltransferase V (Mgat5) is up-regulated in carcinomas and promotes the substitution of N-glycan with poly N-acetyllactosamine, preferred ligand for galectin-3 (Gal-3). Here, we report that expression Mgat5 sensitized mouse cells to multiple cytokines. Gal-3 cross-linked Mgat5-modified N-glycans on epidermal growth factor transforming factor-beta receptors at cell surface delayed their removal by constitutive endocytosis. mammary carcinoma was rate...
Abstract In the developed world, extreme prematurity is leading cause of neonatal mortality and morbidity due to a combination organ immaturity iatrogenic injury. Until now, efforts extend gestation using extracorporeal systems have achieved limited success. Here we report development system that incorporates pumpless oxygenator circuit connected fetus lamb via an umbilical cord interface maintained within closed ‘amniotic fluid’ closely reproduces environment womb. We show fetal lambs are...
Macromolecular complexes exhibit reduced diffusion in biological membranes; however, the physiological consequences of this characteristic plasma membrane domain organization remain elusive. We report that competition between galectin lattice and oligomerized caveolin-1 microdomains for epidermal growth factor (EGF) receptor (EGFR) recruitment regulates EGFR signaling tumor cells. In mammary cells deficient Golgi β1,6N-acetylglucosaminyltransferase V (Mgat5), a reduction binding to allows an...
Soluble transforming growth factor-beta (TGF-beta)/bone morphogenetic protein (BMP)-binding proteins are widely distributed in mammalian tissues and control cytokine access to membrane signaling receptors. The serum bone-resident glycoprotein alpha2-HS-glycoprotein/fetuin (ASHG) binds TGF-beta/BMP cytokines blocks TGF-beta1 binding cell surface Therefore, we examined bone remodeling phenotypes ASHG-deficient mice. skeletal structure of Ahsg(-/-) mice appeared normal at birth, but...
Since the early 1980's, with clinical advent of in vitro fertilization resulting so-called "test tube babies," a wide array ethical considerations and concerns regarding artificial womb technology (AWT) have been described. Recent breakthroughs development extracorporeal neonatal life support by means AWT reinitiated interest about this topic sense urgency. Most recent literature on topic, however, pertains not to more imminent scenario physiologically improved method care through AWT, but...
<b><i>Introduction:</i></b> Randomized controlled trials found that fetoscopic endoluminal tracheal occlusion (FETO) resulted in increased fetal lung volume and improved survival for infants with isolated, severe left-sided congenital diaphragmatic hernia (CDH). The delivery room resuscitation of these is particularly unique, the specific events are largely unknown. objective this study was to compare treated FETO standard care (SOC) describe lessons learned....
Abstract Transforming growth factor (TGF)-β1 is associated with tumor progression and resistance to chemotherapy in established cancers, as well host immune suppression. Here, we show that the serum glycoprotein α2-HS-glycoprotein (AHSG) blocks TGF-β1 binding cell surface receptors, suppresses TGF-β signal transduction, inhibits TGF-β-induced epithelial-mesenchymal transition, suggesting AHSG may play a role progression. In 66 consecutive sporadic human colorectal cancer specimens, observed...
Bronchopulmonary dysplasia is a disease of extreme prematurity that occurs when the immature lung exposed to gas ventilation. We designed novel 'artificial womb' system for supporting premature lambs (called EXTEND) obviates ventilation by providing oxygen via pumpless arteriovenous circuit with lamb submerged in sterile artificial amniotic fluid. In present study, we compare different cannulation strategies on EXTEND, including carotid artery/jugular vein (CA/JV), artery/umbilical (CA/UV)...
Abstract Golgi β1,6N-acetylglucosaminyltransferase V (Mgat5) produces β1,6GlcNAc-branched complex N-glycans on cell surface glycoproteins that bind to galectins and promote residency of glycoproteins, including cytokine receptors. Carcinoma cells from polyomavirus middle T (PyMT) transgenic mice a Mgat5−/− background have reduced levels epidermal growth factor (EGF) transforming factor-β (TGF-β) receptors are less sensitive acute stimulation by cytokines in vitro compared with PyMT Mgat5+/+...
Abstract EXTEND (EXTra‐uterine Environment for Neonatal Development) is a novel system supporting extremely premature infants that replicates in utero conditions by maintaining sterile fluid environment and providing gas exchange via pumpless arteriovenous oxygenator circuit connected to the umbilical vessels. Target gestational age (GA) support human 23‐27 weeks, when immature lungs are most susceptible injury setting of air ventilation. We previously demonstrated physiologic lambs...
Golgi beta1,6-N-acetylglucosaminyltransferase V (Mgat5) produces beta1,6GlcNAc-branched N-glycans on glycoproteins, which increases their affinity for galectins and opposes loss from the cell surface to constitutive endocytosis. Oncogenic transformation Mgat5 expression, epidermal growth factor transforming factor-beta receptors, enhances sensitivities ligands, motility, tumor metastasis. Here, we demonstrate that Mgat5(-/-) mouse embryonic fibroblasts (MEFs) display reduced sensitivity...
Whereas left-sided congenital diaphragmatic hernias (L-CDH) have been extensively studied and their prognostic parameters delineated, right-sided (R-CDH) not. Published results remain inconclusive. The aim of this study is to evaluate if proven indicators postnatal survival in the fetus with L-CDH apply R-CDH.Retrospective single-center R-CDH fetuses available prenatal studies assessed for fetal lung volume by means ultrasound-measured observed versus expected (O/E) area head circumference...