David J. McCulley
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View article: Developmentally Regulated CYP2E1 Expression Is Associated with a Fetal Pulmonary Transcriptional Response to Maternal Acetaminophen Exposure
Developmentally Regulated CYP2E1 Expression Is Associated with a Fetal Pulmonary Transcriptional Response to Maternal Acetaminophen Exposure Open
Background/Objectives: Acetaminophen (APAP) is used during 50–60% of pregnancies in the U.S. and has been associated with childhood respiratory morbidity, though the underlying mechanism remains unclear. APAP-induced injury is dependent on…
View article: MYRF is Essential in Mesothelial Cells to Promote Lung Development and Maturation
MYRF is Essential in Mesothelial Cells to Promote Lung Development and Maturation Open
Summary The mesothelium is a squamous monolayer that ensheathes internal organs, lines the body cavity, and the diaphragm. It serves as a protective barrier, coated in glycocalyx, and secretes lubricants to facilitate tissue movement. How …
View article: Risk Factors Associated With the Development of Late Pulmonary Artery Hypertension in Extremely Premature Infants
Risk Factors Associated With the Development of Late Pulmonary Artery Hypertension in Extremely Premature Infants Open
Objective To identify risk factors for late pulmonary artery hypertension (PH) at 36 weeks' postmenstrual age (PMA) in infants born before 28 weeks' gestation. Design/Methods A retrospective cohort study included infants born < 28 weeks' g…
View article: Mitochondrial Lon Peptidase 1 Controls Diaphragm and Lung Development in a Context-Dependent Manner
Mitochondrial Lon Peptidase 1 Controls Diaphragm and Lung Development in a Context-Dependent Manner Open
Congenital Diaphragmatic Hernia (CDH) is a rare neonatal disorder causing diaphragmatic defects and cardiopulmonary hypoplasia, traditionally attributed to mechanical compression from organ herniation. However, emerging evidence suggests g…
View article: Context-dependent roles of mitochondrial LONP1 in orchestrating the balance between airway progenitor versus progeny cells
Context-dependent roles of mitochondrial LONP1 in orchestrating the balance between airway progenitor versus progeny cells Open
While all eukaryotic cells are dependent on mitochondria for function, in a complex tissue, which cell type and which cell behavior are more sensitive to mitochondrial deficiency remain unpredictable. Here, we show that in the mouse airway…
View article: Rescuing lung development through embryonic inhibition of histone acetylation
Rescuing lung development through embryonic inhibition of histone acetylation Open
A major barrier to the impact of genomic diagnosis in patients with congenital malformations is the lack of understanding regarding how sequence variants contribute to disease pathogenesis and whether this information could be used to gene…
View article: PLS3 missense variants affecting the actin-binding domains cause X-linked congenital diaphragmatic hernia and body-wall defects
PLS3 missense variants affecting the actin-binding domains cause X-linked congenital diaphragmatic hernia and body-wall defects Open
Congenital diaphragmatic hernia (CDH) is a relatively common and genetically heterogeneous structural birth defect associated with high mortality and morbidity. We describe eight unrelated families with an X-linked condition characterized …
View article: Acetaminophen for the patent ductus arteriosus: has safety been adequately demonstrated?
Acetaminophen for the patent ductus arteriosus: has safety been adequately demonstrated? Open
View article: Context-dependent functions of mitochondria protein quality control in lung
Context-dependent functions of mitochondria protein quality control in lung Open
SUMMARY Aside from its role as the universal energy source of the cell, mitochondria also control many aspects of cell behavior. In an intact tissue, whether all cells require mitochondria function to the same extent, and how mitochondria …
View article: Racing against time: leveraging preclinical models to understand pulmonary susceptibility to perinatal acetaminophen exposures
Racing against time: leveraging preclinical models to understand pulmonary susceptibility to perinatal acetaminophen exposures Open
Over the past decade, clinicians have increasingly prescribed acetaminophen (APAP) for patients in the neonatal intensive care unit (NICU). Acetaminophen has been shown to reduce postoperative opiate burden, and may provide similar efficac…
View article: Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene
Rare and de novo variants in 827 congenital diaphragmatic hernia probands implicate LONP1 as candidate risk gene Open
View article: Missense variants affecting the actin-binding domains of <i>PLS3</i> cause X-linked congenital diaphragmatic hernia and body wall defects
Missense variants affecting the actin-binding domains of <i>PLS3</i> cause X-linked congenital diaphragmatic hernia and body wall defects Open
Congenital diaphragmatic hernia (CDH) is a relatively common and genetically heterogeneous structural birth defect associated with high mortality and morbidity. We describe eight unrelated families with a novel X-linked condition character…
View article: Rare and <i>de novo</i> variants in 827 congenital diaphragmatic hernia probands implicate <i>LONP1</i> and <i>ALYREF</i> as new candidate risk genes
Rare and <i>de novo</i> variants in 827 congenital diaphragmatic hernia probands implicate <i>LONP1</i> and <i>ALYREF</i> as new candidate risk genes Open
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that is often accompanied by other anomalies. Although the role of genetics in the pathogenesis of CDH has been established, only a small number of disease genes have bee…
View article: Neurally-Adjusted Ventilatory Assist Can Facilitate Extubation in Neonates With Congenital Diaphragmatic Hernia
Neurally-Adjusted Ventilatory Assist Can Facilitate Extubation in Neonates With Congenital Diaphragmatic Hernia Open
Both invasive ventilation with NAVA and NIV with NAVA were used successfully in subjects with congenital diaphragmatic hernia during the transition from invasive ventilation to NIV. The transition to NAVA was associated with a decrease in …
View article: Likely damaging de novo variants in congenital diaphragmatic hernia patients are associated with worse clinical outcomes
Likely damaging de novo variants in congenital diaphragmatic hernia patients are associated with worse clinical outcomes Open
View article: PBX transcription factors drive pulmonary vascular adaptation to birth
PBX transcription factors drive pulmonary vascular adaptation to birth Open
A critical event in the adaptation to extrauterine life is relaxation of the pulmonary vasculature at birth, allowing for a rapid increase in pulmonary blood flow that is essential for efficient gas exchange. Failure of this transition lea…
View article: Congenital diaphragmatic hernias: from genes to mechanisms to therapies
Congenital diaphragmatic hernias: from genes to mechanisms to therapies Open
Congenital diaphragmatic hernias (CDHs) and structural anomalies of the diaphragm are a common class of congenital birth defects that are associated with significant morbidity and mortality due to associated pulmonary hypoplasia, pulmonary…
View article: Syndactyly in a novel <i>Fras1</i><sup><i>rdf</i></sup> mutant results from interruption of signals for interdigital apoptosis
Syndactyly in a novel <i>Fras1</i><sup><i>rdf</i></sup> mutant results from interruption of signals for interdigital apoptosis Open
Background: Fras1 encodes an extracellular matrix protein that is critical for the establishment of the epidermal basement membrane during gestation. In humans, mutations in FRAS1 cause Fraser Syndrome (FS), a pleiotropic condition with ma…
View article: MEF2C regulates outflow tract alignment and transcriptional control of <i>Tdgf1</i>
MEF2C regulates outflow tract alignment and transcriptional control of <i>Tdgf1</i> Open
Congenital heart defects are the most common birth defects in humans, and defects that affect the proper alignment of the outflow tracts and septation of the ventricles are a highly significant cause of morbidity and mortality in infants. …
View article: The pulmonary mesenchyme directs lung development
The pulmonary mesenchyme directs lung development Open