The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

PJ Moore, R Tarran - Expert opinion on therapeutic targets, 2018 - Taylor & Francis
Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Slippery when wet: airway surface liquid homeostasis and mucus hydration

MJ Webster, R Tarran - Current topics in membranes, 2018 - Elsevier
The ability to regulate cell volume is crucial for normal physiology; equally the regulation of
extracellular fluid homeostasis is of great importance. Alteration of normal extracellular fluid …

A SPLUNC1 peptidomimetic inhibits Orai1 and reduces inflammation in a murine allergic asthma model

JA Wrennall, S Ahmad, EN Worthington… - American Journal of …, 2022 - atsjournals.org
Orai1 is a plasma membrane Ca2+ channel that mediates store-operated Ca2+ entry
(SOCE) and regulates inflammation. Short palate lung and nasal epithelial clone 1 …

Specific Inhibition of Orai1-mediated Calcium Signalling Resolves Inflammation and Clears Bacteria in an Acute Respiratory Distress Syndrome Model

S Ahmad, JA Wrennall, AS Goriounova… - American Journal of …, 2024 - atsjournals.org
Rationale: Acute respiratory distress syndrome (ARDS) has an unacceptably high mortality
rate (35%) and is without effective therapy. Orai1 is a Ca2+ channel involved in store …

SPLUNC1: a novel marker of cystic fibrosis exacerbations

S Khanal, M Webster, N Niu, J Zielonka… - European …, 2021 - Eur Respiratory Soc
Background Acute pulmonary exacerbations (AE) are episodes of clinical worsening in
cystic fibrosis (CF), often precipitated by infection. Timely detection is critical to minimise …

[HTML][HTML] Killing three birds with one BPI: Bactericidal, opsonic, and anti-inflammatory functions

J Theprungsirikul, S Skopelja-Gardner… - Journal of Translational …, 2021 - Elsevier
Bactericidal/permeability-increasing protein (BPI) is an anti-microbial protein predominantly
expressed in azurophilic granules of neutrophils. BPI has been shown to mediate cytocidal …

[HTML][HTML] The effect of BPIFA1/SPLUNC1 genetic variation on its expression and function in asthmatic airway epithelium

N Schaefer, X Li, MA Seibold, NN Jarjour… - JCI insight, 2019 - ncbi.nlm.nih.gov
Bacterial permeability family member A1 (BPIFA1), also known as short palate, lung, and
nasal epithelium clone 1 (SPLUNC1), is a protein involved in the antiinflammatory response …

Association of innate defense proteins BPIFA1 and BPIFB1 with disease severity in COPD

EG De Smet, LJM Seys, FM Verhamme… - … journal of chronic …, 2017 - Taylor & Francis
Chronic obstructive pulmonary disease (COPD) is characterized by an abnormal
inflammatory response in the lungs caused by the inhalation of noxious particles and gases …

Elevated sputum BPIFB1 levels in smokers with chronic obstructive pulmonary disease: a longitudinal study

J Gao, S Ohlmeier, P Nieminen… - … of Physiology-Lung …, 2015 - journals.physiology.org
A previous study involving a proteomic screen of induced sputum from smokers and patients
with chronic obstructive pulmonary disease (COPD) demonstrated elevated levels of …

Mining GWAS and eQTL data for CF lung disease modifiers by gene expression imputation

H Dang, D Polineni, RG Pace, JR Stonebraker… - PloS one, 2020 - journals.plos.org
Genome wide association studies (GWAS) have identified several genomic loci with
candidate modifiers of cystic fibrosis (CF) lung disease, but only a small proportion of the …