[HTML][HTML] The pig as a model for immunology research

R Pabst - Cell and tissue research, 2020 - Springer
The pig is an omnivorous, monogastric species with many advantages to serve as an animal
model for human diseases. There are very high similarities to humans in anatomy and …

Cystic fibrosis: emergence of highly effective targeted therapeutics and potential clinical implications

MA Mall, N Mayer-Hamblett… - American journal of …, 2020 - atsjournals.org
Cystic fibrosis (CF) remains the most common life-shortening hereditary disease in white
populations, with high morbidity and mortality related to chronic airway mucus obstruction …

[HTML][HTML] Pf bacteriophage and their impact on Pseudomonas virulence, mammalian immunity, and chronic infections

PR Secor, EB Burgener, M Kinnersley… - Frontiers in …, 2020 - frontiersin.org
Pf bacteriophage are temperate phages that infect the bacterium Pseudomonas aeruginosa,
a major cause of chronic lung infections in cystic fibrosis (CF) and other settings. Pf and …

Treating cystic fibrosis with mRNA and CRISPR

A Da Silva Sanchez, K Paunovska, A Cristian… - Human gene …, 2020 - liebertpub.com
Less than 20% of the protein coding genome is thought to be targetable using small
molecules. mRNA therapies are not limited in the same way since in theory, they can silence …

[HTML][HTML] Novel anti-inflammatory approaches for cystic fibrosis lung disease: Identification of molecular targets and design of innovative therapies

C Mitri, Z Xu, P Bardin, H Corvol, L Touqui… - Frontiers in …, 2020 - frontiersin.org
Cystic fibrosis (CF) is the most common genetic disorder among Caucasians, estimated to
affect more than 70,000 people in the world. Severe and persistent bronchial inflammation …

Ivacaftor reverses airway mucus abnormalities in a rat model harboring a humanized G551D-CFTR

SE Birket, JM Davis, CM Fernandez-Petty… - American journal of …, 2020 - atsjournals.org
Rationale: Animal models have been highly informative for understanding the
characteristics, onset, and progression of cystic fibrosis (CF) lung disease. In particular, the …

[HTML][HTML] Cystic fibrosis transmembrane conductance regulator dysfunction in platelets drives lung hyperinflammation

G Ortiz-Muñoz, AY Michelle… - The Journal of …, 2020 - Am Soc Clin Investig
Cystic fibrosis (CF) lung disease is characterized by an inflammatory response that can lead
to terminal respiratory failure. The cystic fibrosis transmembrane conductance regulator …

[HTML][HTML] Lack of airway submucosal glands impairs respiratory host defenses

LS Ostedgaard, MP Price, KM Whitworth… - Elife, 2020 - elifesciences.org
Submucosal glands (SMGs) are a prominent structure that lines human cartilaginous
airways. Although it has been assumed that SMGs contribute to respiratory defense, that …

[HTML][HTML] Phenotypic characterization and comparison of cystic fibrosis rat models generated using CRISPR/Cas9 gene editing

A McCarron, P Cmielewski, N Reyne, C McIntyre… - The American journal of …, 2020 - Elsevier
Animal models of cystic fibrosis (CF) are essential for investigating disease mechanisms and
trialing potential therapeutics. This study generated two CF rat models using clustered …

Recombinant acid ceramidase reduces inflammation and infection in cystic fibrosis

AI Gardner, IJ Haq, AJ Simpson, KA Becker… - American Journal of …, 2020 - atsjournals.org
Rationale: In cystic fibrosis the major cause of morbidity and mortality is lung disease
characterized by inflammation and infection. The influence of sphingolipid metabolism is …