Small-molecule correctors of defective ΔF508-CFTR cellular processing identified by high-throughput screening

N Pedemonte, GL Lukacs, K Du, E Caci… - The Journal of …, 2005 - Am Soc Clin Investig
The most common cause of cystic fibrosis (CF) is deletion of phenylalanine 508 (ΔF508) in
the CF transmembrane conductance regulator (CFTR) chloride channel. The ΔF508 …

Dual oxidases represent novel hydrogen peroxide sources supporting mucosal surface host defense

M Geiszt, J Witta, J Baff, K Lekstrom… - The FASEB journal, 2003 - Wiley Online Library
Lactoperoxidase (LPO) is an enzyme with antimicrobial properties present in saliva, milk,
tears, and airway secretions. Although the formation of microbicidal oxidants by LPO has …

Biofilm penetration, triggered release and in vivo activity of inhaled liposomal amikacin in chronic Pseudomonas aeruginosa lung infections

P Meers, M Neville, V Malinin, AW Scotto… - Journal of …, 2008 - academic.oup.com
Abstract Objectives Chronic infections of Pseudomonas aeruginosa in the lungs of cystic
fibrosis patients are intractable antibiotic targets because of their biofilm mode of growth. We …

The mucus barrier to inhaled gene therapy

GA Duncan, J Jung, J Hanes, JS Suk - Molecular Therapy, 2016 - cell.com
Recent evidence suggests that the airway mucus gel layer may be impermeable to the viral
and synthetic gene vectors used in past inhaled gene therapy clinical trials for diseases like …

Aberrant CFTR-dependent HCO-3 transport in mutations associated with cystic fibrosis

JY Choi, D Muallem, K Kiselyov, MG Lee, PJ Thomas… - Nature, 2001 - nature.com
Cystic fibrosis (CF) is a disease caused by mutations in the cystic fibrosis transmembrane
conductance regulator (CFTR). Initially, Cl-conductance in the sweat duct was discovered to …

Defective lipoxin-mediated anti-inflammatory activity in the cystic fibrosis airway

CL Karp, LM Flick, KW Park, S Softic, TM Greer… - Nature …, 2004 - nature.com
In cystic fibrosis, dysregulated neutrophilic inflammation and chronic infection lead to
progressive destruction of the airways. The underlying mechanisms have remained unclear …

State of the art: why do the lungs of patients with cystic fibrosis become infected and why can't they clear the infection?

JF Chmiel, PB Davis - Respiratory research, 2003 - Springer
Cystic Fibrosis (CF) lung disease, which is characterized by airway obstruction, chronic
bacterial infection, and an excessive inflammatory response, is responsible for most of the …

The biological potential of flavones

AK Verma, R Pratap - Natural product reports, 2010 - pubs.rsc.org
Covering: 1990 to 2009 Flavones are well known for their antioxidant activity, and are
unique in that they are present in the majority of fruits and vegetables. The majority of …

[HTML][HTML] A survey of newborn screening for cystic fibrosis in Europe

KW Southern, A Munck, R Pollitt, G Travert… - Journal of Cystic …, 2007 - Elsevier
BACKGROUND: Cystic fibrosis (CF) is a recessively inherited condition caused by mutation
of the CFTR gene. Newborn infants with CF have raised levels of immuno-reactive …

A novel host defense system of airways is defective in cystic fibrosis

P Moskwa, D Lorentzen, KJDA Excoffon… - American journal of …, 2007 - atsjournals.org
Rationale: The respiratory tract is constantly exposed to airborne microorganisms.
Nevertheless, normal airways remain sterile without recruiting phagocytes. This innate …