[HTML][HTML] Tubular lipid binding proteins (TULIPs) growing everywhere

LH Wong, TP Levine - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2017 - Elsevier
Tubular lipid binding proteins (TULIPs) have become a focus of interest in the cell biology of
lipid signalling, lipid traffic and membrane contact sites. Each tubular domain has an internal …

Bactericidal/permeability-increasing protein fold–containing family member A1 in airway host protection and respiratory disease

CJ Britto, L Cohn - American journal of respiratory cell and …, 2015 - atsjournals.org
Bactericidal/permeability-increasing protein fold–containing family member A1 (BPIFA1),
formerly known as SPLUNC1, is one of the most abundant proteins in respiratory secretions …

SPX-101 is a novel epithelial sodium channel–targeted therapeutic for cystic fibrosis that restores mucus transport

DW Scott, MP Walker, J Sesma, B Wu… - American journal of …, 2017 - atsjournals.org
Rationale: Cystic fibrosis (CF) lung disease is caused by the loss of function of the cystic
fibrosis transmembrane conductance regulator (CFTR) combined with hyperactivation of the …

[HTML][HTML] Identification of BPIFA1/SPLUNC1 as an epithelium-derived smooth muscle relaxing factor

T Wu, J Huang, PJ Moore, MS Little, WG Walton… - Nature …, 2017 - nature.com
Asthma is a chronic airway disease characterized by inflammation, mucus hypersecretion
and abnormal airway smooth muscle (ASM) contraction. Bacterial permeability family …

Effects of airway surface liquid pH on host defense in cystic fibrosis

AR Berkebile, PB McCray Jr - The international journal of biochemistry & …, 2014 - Elsevier
Cystic fibrosis is a lethal genetic disorder characterized by viscous mucus and bacterial
colonization of the airways. Airway surface liquid represents a first line of pulmonary …

Biofilm formations in pediatric respiratory tract infection: part 1: biofilm structure, role of innate immunity in protection against and response to biofilm, methods of …

DL Hamilos - Current infectious disease reports, 2019 - Springer
Abstract Purpose of Review Biofilm represents an organized structure of microorganisms
within an extracellular matrix attached to a surface. While the importance of biofilm in …

The microbiota, chemical symbiosis, and human disease

MR Redinbo - Journal of Molecular Biology, 2014 - Elsevier
Our understanding of mammalian–microbial mutualism has expanded by combing microbial
sequencing with evolving molecular and cellular methods, as well as unique model systems …

Viral vectors, animal models, and cellular targets for gene therapy of cystic fibrosis lung disease

Y Tang, Z Yan, JF Engelhardt - Human gene therapy, 2020 - liebertpub.com
After more than two decades since clinical trials tested the first use of recombinant adeno-
associated virus (rAAV) to treat cystic fibrosis (CF) lung disease, gene therapy for this …

Choline in cystic fibrosis: relations to pancreas insufficiency, enterohepatic cycle, PEMT and intestinal microbiota

W Bernhard - European journal of nutrition, 2021 - Springer
Abstract Background Cystic Fibrosis (CF) is an autosomal recessive disorder with life-
threatening organ manifestations. 87% of CF patients develop exocrine pancreas …

SPLUNC1 degradation by the cystic fibrosis mucosal environment drives airway surface liquid dehydration

MJ Webster, B Reidel, CD Tan, A Ghosh… - European …, 2018 - Eur Respiratory Soc
The multi-organ disease cystic fibrosis (CF) is caused by mutations in the cystic fibrosis
transmembrane regulator gene (CFTR) that lead to diminished transepithelial anion …