Mucus, mucins, and cystic fibrosis

CB Morrison, MR Markovetz, C Ehre - Pediatric pulmonology, 2019 - Wiley Online Library
Cystic fibrosis (CF) is both the most common and most lethal genetic disease in the
Caucasian population. CF is caused by mutations in the cystic fibrosis transmembrane …

Neutrophil adaptations upon recruitment to the lung: new concepts and implications for homeostasis and disease

VD Giacalone, C Margaroli, MA Mall… - International journal of …, 2020 - mdpi.com
Neutrophils have a prominent role in all human immune responses against any type of
pathogen or stimulus. The lungs are a major neutrophil reservoir and neutrophilic …

Progression of cystic fibrosis lung disease from childhood to adulthood: neutrophils, neutrophil extracellular trap (NET) formation, and NET degradation

MA Khan, ZS Ali, N Sweezey, H Grasemann… - Genes, 2019 - mdpi.com
Genetic defects in cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene
cause CF. Infants with CFTR mutations show a peribronchial neutrophil infiltration prior to …

Elastase exocytosis by airway neutrophils is associated with early lung damage in children with cystic fibrosis

C Margaroli, LW Garratt, H Horati… - American Journal of …, 2019 - atsjournals.org
Rationale: Neutrophils are recruited to the airways of individuals with cystic fibrosis (CF). In
adolescents and adults with CF, airway neutrophils actively exocytose the primary granule …

Transcriptional reprogramming of infiltrating neutrophils drives lung pathology in severe COVID-19 despite low viral load

DJ Eddins, J Yang, A Kosters, VD Giacalone… - Blood …, 2023 - ashpublications.org
Troubling disparities in COVID-19–associated mortality emerged early, with nearly 70% of
deaths confined to Black/African American (AA) patients in some areas. However, targeted …

Dysregulated signalling pathways in innate immune cells with cystic fibrosis mutations

S Lara-Reyna, J Holbrook, HH Jarosz-Griffiths… - Cellular and Molecular …, 2020 - Springer
Cystic fibrosis (CF) is one of the most common life-limiting recessive genetic disorders in
Caucasians, caused by mutations in the cystic fibrosis transmembrane conductance …

Methionine sulfoxide and the methionine sulfoxide reductase system as modulators of signal transduction pathways: a review

J Moskovitz, A Smith - Amino Acids, 2021 - Springer
Methionine oxidation and reduction is a common phenomenon occurring in biological
systems under both physiological and oxidative-stress conditions. The levels of methionine …

Transcriptional firing represses bactericidal activity in cystic fibrosis airway neutrophils

C Margaroli, D Moncada-Giraldo, DA Gulick… - Cell Reports …, 2021 - cell.com
Neutrophils are often considered terminally differentiated and poised for bacterial killing. In
chronic diseases such as cystic fibrosis (CF), an unexplained paradox pits massive …

CFTR correctors and antioxidants partially normalize lipid imbalance but not abnormal basal inflammatory cytokine profile in CF bronchial epithelial cells

M Veltman, JB De Sanctis, M Stolarczyk… - Frontiers in …, 2021 - frontiersin.org
A deficiency in cystic fibrosis transmembrane conductance regulator (CFTR) function in CF
leads to chronic lung disease. CF is associated with abnormalities in fatty acids, ceramides …

Low-dose vanadium pentoxide perturbed lung metabolism associated with inflammation and fibrosis signaling in male animal and in vitro models

X He, ZR Jarrell, MR Smith, VLT Ly… - … of Physiology-Lung …, 2023 - journals.physiology.org
Vanadium is available as a dietary supplement and also is known to be toxic if inhaled, yet
little information is available concerning the effects of vanadium on mammalian metabolism …