(Q34671320)

3 August 2017

Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia (English)

1 reference

3 August 2017

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8

Leah R Reznikov

1 reference

3 August 2017

Jeng-Haur Chen

1

1 reference

3 August 2017

David A Stoltz

2

1 reference

3 August 2017

Philip H Karp

3

1 reference

3 August 2017

Sarah E Ernst

4

1 reference

3 August 2017

Alejandro A Pezzulo

5

1 reference

3 August 2017

Thomas O Moninger

6

1 reference

3 August 2017

Michael V Rector

7

1 reference

3 August 2017

Janice L Launspach

9

1 reference

3 August 2017

Kathryn Chaloner

10

1 reference

3 August 2017

Joseph Zabner

11

1 reference

3 August 2017

Michael J Welsh

12

1 reference

3 August 2017

language of work or name

English

0 references

publication date

1 December 2010

1 reference

3 August 2017

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3 August 2017

143

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3 August 2017

911-923

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3 August 2017

6

1 reference

describes a project that uses

3 August 2017

ImageJ

1 reference

11 June 2022

https://www.ebi.ac.uk/europepmc/webservices/rest/PMC3057187/fullTextXML

inferred from PubMed Central ID database lookup

cites work

Loss of cystic fibrosis transmembrane conductance regulator function produces abnormalities in tracheal development in neonatal pigs and young children

1 reference

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Cystic fibrosis pigs develop lung disease and exhibit defective bacterial eradication at birth

7 July 2018

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Pathology of gastrointestinal organs in a porcine model of cystic fibrosis

7 July 2018

1 reference

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Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770

7 July 2018

1 reference

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Acute regulation of tight junction ion selectivity in human airway epithelia

7 July 2018

1 reference

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Disruption of the CFTR gene produces a model of cystic fibrosis in newborn pigs

7 July 2018

1 reference

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Production of CFTR-null and CFTR-DeltaF508 heterozygous pigs by adeno-associated virus-mediated gene targeting and somatic cell nuclear transfer

7 July 2018

1 reference

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Sodium channels and cystic fibrosis

7 July 2018

1 reference

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Processing and function of CFTR-DeltaF508 are species-dependent

7 July 2018

1 reference

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Cystic Fibrosis: Lessons from the Sweat Gland

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Airway surface dehydration in cystic fibrosis: pathogenesis and therapy

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Role of Ca2+ in responses of airway epithelia to Pseudomonas aeruginosa, flagellin, ATP, and thapsigargin

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Cystic fibrosis since 1938.

7 July 2018

1 reference

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7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Potential difference measurements in the lower airway of children with and without cystic fibrosis.

7 July 2018

1 reference

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Discovery of glycine hydrazide pore-occluding CFTR inhibitors: mechanism, structure-activity analysis, and in vivo efficacy

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Standardized procedure for measurement of nasal potential difference: an outcome measure in multicenter cystic fibrosis clinical trials

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

STUDIES ON CILIA. THE FIXATION OF THE METACHRONAL WAVE

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

ENaC-CFTR interactions: the role of electrical coupling of ion fluxes explored in an epithelial cell model

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Lung symptoms in pseudohypoaldosteronism type 1 are associated with deficiency of the alpha-subunit of the epithelial sodium channel

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

The genesis of cystic fibrosis lung disease

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Pathophysiology of gene-targeted mouse models for cystic fibrosis

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Structure and function of the CFTR chloride channel

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

The amiloride-inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways.

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Abnormalities of nasal potential difference measurement in Liddle's syndrome

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Effect of increasing doses of hypertonic saline on mucociliary clearance in patients with cystic fibrosis

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Heterogeneity of the composition and thickness of tracheal mucus in rats.

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Bicarbonate conductance and pH regulatory capability of cystic fibrosis transmembrane conductance regulator

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Relationship of a non-cystic fibrosis transmembrane conductance regulator-mediated chloride conductance to organ-level disease in Cftr(-/-) mice

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Cystic fibrosis decreases the apical membrane chloride permeability of monolayers cultured from cells of tracheal epithelium

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Na+ transport in cystic fibrosis respiratory epithelia. Abnormal basal rate and response to adenylate cyclase activation

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Evidence for reduced Cl- and increased Na+ permeability in cystic fibrosis human primary cell cultures.

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Regulation of sodium absorption by canine tracheal epithelium.

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Transcellular sodium transport in cultured cystic fibrosis human nasal epithelium

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Sodium transport and intracellular sodium activity in cultured human nasal epithelium.

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

An analytical model of ionic movements in airway epithelial cells

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

cAMP stimulates bicarbonate secretion across normal, but not cystic fibrosis airway epithelia

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Quantification of periciliary fluid height in human airway biopsies is feasible, but not suitable as a biomarker

7 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Functional characterization of a partial loss-of-function mutation of the epithelial sodium channel (ENaC) associated with atypical cystic fibrosis.

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Mutations in the amiloride-sensitive epithelial sodium channel in patients with cystic fibrosis-like disease.

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Airway surface liquid volume regulates ENaC by altering the serine protease-protease inhibitor balance: a mechanism for sodium hyperabsorption in cystic fibrosis.

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Mucus clearance and lung function in cystic fibrosis with hypertonic saline

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

A controlled trial of long-term inhaled hypertonic saline in patients with cystic fibrosis

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Flagellin of Pseudomonas aeruginosa inhibits Na+ transport in airway epithelia

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Hyposecretion, not hyperabsorption, is the basic defect of cystic fibrosis airway glands

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Mutations in the beta-subunit of the epithelial Na+ channel in patients with a cystic fibrosis-like syndrome

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Regulation of MUC5AC mucin secretion and airway surface liquid metabolism by IL-1β in human bronchial epithelia

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

An in vitro model of differentiated human airway epithelia. Methods for establishing primary cultures.

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Physiological basis of cystic fibrosis: a historical perspective

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Loss of CFTR chloride channels alters salt absorption by cystic fibrosis airway epithelia in vitro.

26 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3057187

Edge damage effect on electrical measurements of frog skin

26 October 2018

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21145458

12 December 2020

inferred from PubMed ID database lookup

Increased bioelectric potential difference across respiratory epithelia in cystic fibrosis

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21145458

12 December 2020

inferred from PubMed ID database lookup

Abnormal Ion Permeation Through Cystic Fibrosis Respiratory Epithelium

1 reference

PubMed

https://pubmed.ncbi.nlm.nih.gov/21145458

12 December 2020

inferred from PubMed ID database lookup

Identifiers

DOI

10.1016/J.CELL.2010.11.029

1 reference

3 August 2017

1 reference

3 August 2017

1 reference