Gene and cell therapy for cystic fibrosis: from bench to bedside

M Conese, F Ascenzioni, AC Boyd, C Coutelle… - Journal of Cystic …, 2011 - Elsevier
Clinical trials in cystic fibrosis (CF) patients established proof-of-principle for transfer of the
wild-type cystic fibrosis transmembrane conductance regulator (CFTR) gene to airway …

Functional roles of SPLUNC1 in the innate immune response against Gram-negative bacteria

YP Di - Biochemical Society Transactions, 2011 - portlandpress.com
PLUNC (palate, lung and nasal epithelium clone)-associated gene originally referred to one
gene, but now has been extended to represent a gene family that consists of a number of …

Novel role for pendrin in orchestrating bicarbonate secretion in cystic fibrosis transmembrane conductance regulator (CFTR)-expressing airway serous cells

JP Garnett, E Hickman, R Burrows, P Hegyi… - Journal of Biological …, 2011 - ASBMB
In most HCO 3−-secreting epithelial tissues, SLC26 Cl−/HCO 3− transporters work in concert
with the cystic fibrosis transmembrane conductance regulator (CFTR) to regulate the …

Single HA2 mutation increases the infectivity and immunogenicity of a live attenuated H5N1 intranasal influenza vaccine candidate lacking NS1

BM Krenn, A Egorov, E Romanovskaya-Romanko… - PLoS …, 2011 - journals.plos.org
Background H5N1 influenza vaccines, including live intranasal, appear to be relatively less
immunogenic compared to seasonal analogs. The main influenza virus surface glycoprotein …

Human bronchial epithelial cells differentiate to 3D glandular acini on basement membrane matrix

X Wu, JR Peters-Hall, S Bose, MT Pena… - American journal of …, 2011 - atsjournals.org
To create a model system that investigates mechanisms resulting in hyperplasia and
hypertrophy of respiratory tract submucosal glands, we developed an in vitro three …

Defective Fluid Secretion from Submucosal Glands of Nasal Turbinates from CFTR-/- and CFTRΔF508/ΔF508 Pigs

HJ Cho, NS Joo, JJ Wine - PloS one, 2011 - journals.plos.org
Background Cystic fibrosis (CF), caused by reduced CFTR function, includes severe
sinonasal disease which may predispose to lung disease. Newly developed CF pigs provide …

Cystic fibrosis and the relationship between mucin and chloride secretion by cultures of human airway gland mucous cells

WE Finkbeiner, LT Zlock, M Morikawa… - … of Physiology-Lung …, 2011 - journals.physiology.org
—We investigated how cystic fibrosis (CF) alters the relationship between Cl− and mucin
secretion in cultures of non-CF and CF human tracheobronchial gland mucous (HTGM and …

CGRP induction in cystic fibrosis airways alters the submucosal gland progenitor cell niche in mice

W Xie, JT Fisher, TJ Lynch, M Luo… - The Journal of …, 2011 - Am Soc Clin Investig
In cystic fibrosis (CF), a lack of functional CF transmembrane conductance regulator (CFTR)
chloride channels causes defective secretion by submucosal glands (SMGs), leading to …

Autonomic neural control of intrathoracic airways

BJ Undem, C Potenzieri - Comprehensive Physiology, 2011 - Wiley Online Library
Autonomic neural control of the intrathoracic airways aids in optimizing air flow and gas
exchange. In addition, and perhaps more importantly, the autonomic nervous system …

Cystic fibrosis and the innate immune system: therapeutic implications

M Conese - Endocrine, Metabolic & Immune Disorders-Drug …, 2011 - ingentaconnect.com
Cystic Fibrosis (CF), the most common autosomal lethal disorder in Caucasians, is caused
by mutations in the CF transmembrane conductance regulator (CFTR) gene. Although CF is …