Respiratory tract mucin genes and mucin glycoproteins in health and disease

MC Rose, JA Voynow - Physiological reviews, 2006 - journals.physiology.org
This review focuses on the role and regulation of mucin glycoproteins (mucins) in airway
health and disease. Mucins are highly glycosylated macromolecules (≥ 50% carbohydrate …

Mouse models of cystic fibrosis: phenotypic analysis and research applications

M Wilke, RM Buijs-Offerman, J Aarbiou… - Journal of Cystic …, 2011 - Elsevier
Genetically modified mice have been studied for more than fifteen years as models of cystic
fibrosis (CF). The large amount of experimental data generated illuminates the complex …

[HTML][HTML] p73-deficient mice have neurological, pheromonal and inflammatory defects but lack spontaneous tumours

A Yang, N Walker, R Bronson, M Kaghad… - Nature, 2000 - nature.com
Abstract p73 (ref.) has high homology with the tumour suppressor p53 (refs,,), as well as with
p63, a gene implicated in the maintenance of epithelial stem cells,,. Despite the localization …

[HTML][HTML] Single-molecule studies of the effect of template tension on T7 DNA polymerase activity

GJL Wuite, SB Smith, M Young, D Keller, C Bustamante - Nature, 2000 - nature.com
Abstract T7 DNA polymerase, catalyses DNA replication in vitro at rates of more than 100
bases per second and has a 3′→ 5′ exonuclease (nucleotide removing) activity at a …

Cystic fibrosis mouse models

C Guilbault, Z Saeed, GP Downey… - American journal of …, 2007 - atsjournals.org
Animal models of cystic fibrosis (CF) are powerful tools that enable the study of the
mechanisms and complexities of human disease. Murine models have several intrinsic …

The NLRP1 inflammasome attenuates colitis and colitis-associated tumorigenesis

TM Williams, RA Leeth, DE Rothschild… - The Journal of …, 2015 - journals.aai.org
Nucleotide-binding domain and leucine-rich repeat (NLR) proteins are a diverse family of
pattern recognition receptors that are essential mediators of inflammation and host defense …

Cell to cell communication in response to mechanical stress via bilateral release of ATP and UTP in polarized epithelia

L Homolya, TH Steinberg, RC Boucher - The Journal of cell biology, 2000 - rupress.org
Airway epithelia are positioned at the interface between the body and the environment, and
generate complex signaling responses to inhaled toxins and other stresses. Luminal …

Purinergic signaling in the airways

G Burnstock, I Brouns, D Adriaensen… - Pharmacological …, 2012 - ASPET
Evidence for a significant role and impact of purinergic signaling in normal and diseased
airways is now beyond dispute. The present review intends to provide the current state of …

Murine models of acute and chronic lung infection with cystic fibrosis pathogens

A Bragonzi - International journal of medical microbiology, 2010 - Elsevier
Animal models of acute and chronic infection, along with mice genetically modified for the
Cftr gene, are a key asset in cystic fibrosis (CF) research. Despite some limitations, these …

[HTML][HTML] Animal models of cystic fibrosis

BJ Scholte, DJ Davidson, M Wilke… - Journal of Cystic Fibrosis, 2004 - Elsevier
Animal models of cystic fibrosis, in particular several different mutant mouse strains obtained
by homologous recombination, have contributed considerably to our understanding of CF …