[HTML][HTML] Innate immunity in cystic fibrosis lung disease

D Hartl, A Gaggar, E Bruscia, A Hector, V Marcos… - Journal of Cystic …, 2012 - Elsevier
Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients.
The pulmonary immune response in CF is characterized by an early and non-resolving …

The role of neutrophils in cystic fibrosis

AM Gifford, JD Chalmers - Current opinion in hematology, 2014 - journals.lww.com
The role of neutrophils in cystic fibrosis : Current Opinion in Hematology The role of neutrophils in
cystic fibrosis : Current Opinion in Hematology Log in or Register Subscribe to journalSubscribe …

Mammalian target of rapamycin regulates neutrophil extracellular trap formation via induction of hypoxia-inducible factor 1 α

AM McInturff, MJ Cody, EA Elliott… - Blood, The Journal …, 2012 - ashpublications.org
Neutrophils are highly specialized innate immune effector cells that evolved for antimicrobial
host defense. In response to inflammatory stimuli and pathogens, they form neutrophil …

Neutrophil Extracellular Trap (NET)-Mediated Killing of Pseudomonas aeruginosa: Evidence of Acquired Resistance within the CF Airway, Independent of CFTR

RL Young, KC Malcolm, JE Kret, SM Caceres… - PloS one, 2011 - journals.plos.org
The inability of neutrophils to eradicate Pseudomonas aeruginosa within the cystic fibrosis
(CF) airway eventually results in chronic infection by the bacteria in nearly 80 percent of …

A neutrophil intrinsic impairment affecting Rab27a and degranulation in cystic fibrosis is corrected by CFTR potentiator therapy

K Pohl, E Hayes, J Keenan, M Henry… - Blood, The Journal …, 2014 - ashpublications.org
Studies have endeavored to reconcile whether dysfunction of neutrophils in people with
cystic fibrosis (CF) is a result of the genetic defect or is secondary due to infection and …

Immune responses in cystic fibrosis: are they intrinsically defective?

D Ratner, C Mueller - American journal of respiratory cell and …, 2012 - atsjournals.org
Cystic fibrosis (CF), the most common lethal single-gene disorder affecting Northern
Europeans and North Americans, is caused by mutations in the cystic fibrosis …

Cystic fibrosis transmembrane conductance regulator recruitment to phagosomes in neutrophils

Y Zhou, K Song, RG Painter, M Aiken, J Reiser… - Journal of innate …, 2013 - karger.com
Optimal microbicidal activity of human polymorphonuclear leukocytes (PMN) relies on the
generation of toxic agents such as hypochlorous acid (HOCl) in phagosomes. HOCl …

Pouring Salt on a Wound: Pseudomonas aeruginosa Virulence Factors Alter Na+ and Cl Flux in the Lung

AE Ballok, GA O'Toole - Journal of bacteriology, 2013 - Am Soc Microbiol
Pseudomonas aeruginosa is a ubiquitous opportunistic pathogen with multiple niches in the
human body, including the lung. P. aeruginosa infections are particularly damaging or fatal …

Aspergillus colonization and antifungal immunity in cystic fibrosis patients

A Warris, A Bercusson, D Armstrong-James - Medical mycology, 2019 - academic.oup.com
Cystic fibrosis (CF), caused by mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) gene, is the most common inherited life-limiting disease in North European …

Altered degranulation and pH of neutrophil phagosomes impacts antimicrobial efficiency in cystic fibrosis

E Hayes, MP Murphy, K Pohl, N Browne… - Frontiers in …, 2020 - frontiersin.org
Studies have endeavored to understand the cause for impaired antimicrobial killing by
neutrophils of people with cystic fibrosis (PWCF). The aim of this study was to focus on the …