Correction of the ion transport defect in cystic fibrosis transgenic mice by gene therapy
…, DR Gill, CF Higgins, AEO Trezise, LJ MacVinish… - Nature, 1993 - nature.com
CYSTIC fibrosis (CF) is a lethal inherited disorder affecting about 1 in 2,000 Caucasians.
The major cause of morbidity is permanent lung damage resulting from ion transport …
The major cause of morbidity is permanent lung damage resulting from ion transport …
Production of a severe cystic fibrosis mutation in mice by gene targeting
R Ratcliff, MJ Evans, AW Cuthbert, LJ MacVinish… - Nature …, 1993 - nature.com
We have used gene targeting in embryonic stem cells to introduce an HPRT mini–gene into
the coding sequence of the murine cystic fibrosis gene (cftr). This insertion introduces a …
the coding sequence of the murine cystic fibrosis gene (cftr). This insertion introduces a …
Generation and characterization of a ΔF508 cystic fibrosis mouse model
…, R Ratcliff, C Jiang, SH Cheng, LJ MacVinish… - Nature …, 1995 - nature.com
We have generated mice carrying the most common mutation in cystic fibrosis (CF), ΔF508,
within the cystic fibrosis (Cftr) gene. Mutant animals show pathological and …
within the cystic fibrosis (Cftr) gene. Mutant animals show pathological and …
A placebo-controlled study of liposome-mediated gene transfer to the nasal epithelium of patients with cystic fibrosis
…, L Huang, F Sorgi, A Thomson, LJ MacVinish… - Gene therapy, 1997 - nature.com
Cystic fibrosis (CF) is a common, serious, inherited disease. The major cause of mortality in
CF is lung disease, due to the failure of airway epithelial cells to express a functional product …
CF is lung disease, due to the failure of airway epithelial cells to express a functional product …
Chloride secretion in response to guanylin in colonic epithelial from normal and transgenic cystic fibrosis mice.
AW Cuthbert, ME Hickman, LJ MacVinish… - British journal of …, 1994 - ncbi.nlm.nih.gov
Guanylin, a 15 amino acid endogenous gut peptide, increased the short circuit current (SCC)
in the epithelium of the mouse colon, but only when applied to the apical and not the …
in the epithelium of the mouse colon, but only when applied to the apical and not the …
Importance of basolateral K+ conductance in maintaining Cl− secretion in murine nasal and colonic epithelia
LJ MacVinish, ME Hickman, DAH Mufti… - The Journal of …, 1998 - Wiley Online Library
1 Epithelia lining the nasal passages and descending colon of wild‐type and cystic fibrosis (CF)
mice were examined by the short‐circuit current technique. Additionally, intracellular Ca …
mice were examined by the short‐circuit current technique. Additionally, intracellular Ca …
[HTML][HTML] Complementation of null CF mice with a human CFTR YAC transgene
AL Manson, AEO Trezise, LJ MacVinish… - The EMBO …, 1997 - embopress.org
We have made transgenic mice carrying a 320 kb YAC with the intact human cystic fibrosis
transmembrane regulator (CFTR) gene. Mice that only express the human transgene were …
transmembrane regulator (CFTR) gene. Mice that only express the human transgene were …
Activation of Ca2+- and cAMP-sensitive K+ channels in murine colonic epithelia by 1-ethyl-2-benzimidazolone
…, P Thorn, LJ MacVinish - American Journal of …, 1999 - journals.physiology.org
1-Ethyl-2-benzimidazolone (EBIO) caused a sustained increase in electrogenic Cl − secretion
in isolated mouse colon mucosae, an effect reduced by blocking basolateral K + channels. …
in isolated mouse colon mucosae, an effect reduced by blocking basolateral K + channels. …
Chloride transporting capability of Calu‐3 epithelia following persistent knockdown of the cystic fibrosis transmembrane conductance regulator, CFTR
LJ MacVinish, G Cope, A Ropenga… - British journal of …, 2007 - Wiley Online Library
Background and purpose: Calu‐3 cells are derived from serous cells of human lung submucosal
glands, a prime target for therapy in cystic fibrosis (CF). Calu‐3 cells can be cultured to …
glands, a prime target for therapy in cystic fibrosis (CF). Calu‐3 cells can be cultured to …
[HTML][HTML] Intra-amniotic delivery of CFTR-expressing adenovirus does not reverse cystic fibrosis phenotype in inbred CFTR-knockout mice
…, S Jezzard, A Bergau, M Themis, LJ MacVinish… - Molecular Therapy, 2008 - cell.com
Due to its early onset and severe prognosis, cystic fibrosis (CF) has been suggested as a
candidate disease for in utero gene therapy. In 1997, a study was published claiming that to …
candidate disease for in utero gene therapy. In 1997, a study was published claiming that to …