(Q42651187)

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A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition

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scholarly article

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Europe PubMed Central

PubMed publication ID

4 November 2017

A mutation of the epithelial sodium channel associated with atypical cystic fibrosis increases channel open probability and reduces Na+ self inhibition (English)

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

cystic fibrosis

0 references

Heinrich Sticht

9

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Abul Kalam Azad

object named as

Abul Kalam Azad

5

0 references

object named as

Robert Rauh

1

0 references

author name string

Alexei Diakov

2

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Anja Tzschoppe

3

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Judit Korbmacher

4

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Harry Cuppens

6

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Jean-Jaques Cassiman

7

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Jörg Dötsch

8

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Christoph Korbmacher

10

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

language of work or name

0 references

publication date

1 March 2010

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Journal of Physiology

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

588

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

Pt 8

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

1211-1225

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

The delta-subunit of the epithelial sodium channel (ENaC) enhances channel activity and alters proteolytic ENaC activation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Pore architecture and ion sites in acid-sensing ion channels and P2X receptors

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

ENaC at the cutting edge: regulation of epithelial sodium channels by proteases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Activation of the epithelial sodium channel (ENaC) by serine proteases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Preventive but not late amiloride therapy reduces morbidity and mortality of lung disease in betaENaC-overexpressing mice.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Epithelial sodium channel: mendelian versus essential hypertension

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Cleavage in the {gamma}-subunit of the epithelial sodium channel (ENaC) plays an important role in the proteolytic activation of near-silent channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Trypsin can activate the epithelial sodium channel (ENaC) in microdissected mouse distal nephron.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Proteolytic processing of the epithelial sodium channel gamma subunit has a dominant role in channel activation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Insight toward epithelial Na+ channel mechanism revealed by the acid-sensing ion channel 1 structure

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Structure of acid-sensing ion channel 1 at 1.9 A resolution and low pH

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Role of proteolysis in the activation of epithelial sodium channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

(NDRG2) stimulates amiloride-sensitive Na+ currents in Xenopus laevis oocytes and fisher rat thyroid cells.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Cystic fibrosis: a disease of vulnerability to airway surface dehydration.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Functional characterization of a novel CFTR mutation P67S identified in a patient with atypical cystic fibrosis.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Epithelial Na+ channels are fully activated by furin- and prostasin-dependent release of an inhibitory peptide from the gamma-subunit.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

A novel neutrophil elastase inhibitor prevents elastase activation and surface cleavage of the epithelial sodium channel expressed in Xenopus laevis oocytes.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Stimulation of the epithelial sodium channel (ENaC) by the serum- and glucocorticoid-inducible kinase (Sgk) involves the PY motifs of the channel but is independent of sodium feedback inhibition

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Cystic fibrosis: terminology and diagnostic algorithms

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Functional polymorphisms in the alpha-subunit of the human epithelial Na+ channel increase activity.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Impact of Nedd4 proteins and serum and glucocorticoid-induced kinases on epithelial Na+ transport in the distal nephron

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Neutrophil elastase activates near-silent epithelial Na+ channels and increases airway epithelial Na+ transport.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

A novel pathway of epithelial sodium channel activation involves a serum- and glucocorticoid-inducible kinase consensus motif in the C terminus of the channel's alpha-subunit

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

New concepts of the pathogenesis of cystic fibrosis lung disease

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Extracellular Na+ removal attenuates rundown of the epithelial Na+-channel (ENaC) by reducing the rate of channel retrieval

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

The role of Pre-H2 domains of alpha- and delta-epithelial Na+ channels in ion permeation, conductance, and amiloride sensitivity

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Serine protease activation of near-silent epithelial Na+ channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Na self inhibition of human epithelial Na channel: temperature dependence and effect of extracellular proteases

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Epithelial sodium channel/degenerin family of ion channels: a variety of functions for a shared structure.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Hormonal regulation of the epithelial sodium channel ENaC: N or P(o)?

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Regulation of the epithelial sodium channel by N4WBP5A, a novel Nedd4/Nedd4-2-interacting protein

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Sulfonylurea receptors inhibit the epithelial sodium channel (ENaC) by reducing surface expression

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

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

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

A new ER trafficking signal regulates the subunit stoichiometry of plasma membrane K(ATP) channels

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Structure and function of the CFTR chloride channel

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Mutations causing Liddle syndrome reduce sodium-dependent downregulation of the epithelial sodium channel in the Xenopus oocyte expression system.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Protease modulation of the activity of the epithelial sodium channel expressed in Xenopus oocytes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Epithelial sodium channels: function, structure, and regulation

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

The ENaC channel as the primary determinant of two human diseases: Liddle syndrome and pseudohypoaldosteronism

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Cell surface expression of the epithelial Na channel and a mutant causing Liddle syndrome: a quantitative approach

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Early death due to defective neonatal lung liquid clearance in alpha-ENaC-deficient mice.

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Trypsin induces Ca(2+)-activated Cl- currents in X. laevis oocytes

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Amiloride-sensitive epithelial Na+ channel is made of three homologous subunits

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

A mutation in the epithelial sodium channel causing Liddle disease increases channel activity in the Xenopus laevis oocyte expression system

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

A calcium-activated and nucleotide-sensitive nonselective cation channel in M-1 mouse cortical collecting duct cells

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Molecular cloning and functional expression of a novel amiloride-sensitive Na+ channel

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

30 September 2018

The TNFα receptor TNFRSF1A and genes encoding the amiloride-sensitive sodium channel ENaC as modulators in cystic fibrosis

1 reference

https://api.crossref.org/works/10.1113%2FJPHYSIOL.2009.180224

21 January 2018

Reporting ethical matters in the Journal of Physiology: standards and advice

1 reference

https://api.crossref.org/works/10.1113%2FJPHYSIOL.2009.180224

21 January 2018

Functional Characterization of a Novel CFTR Mutation P67S Identified in a Patient with Atypical Cystic Fibrosis

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2872728

1 December 2018

beta-Liddle mutation of the epithelial sodium channel increases alveolar fluid clearance and reduces the severity of hydrostatic pulmonary oedema in mice

1 reference

https://pubmed.ncbi.nlm.nih.gov/20194130

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Structural biology: Unexpected opening

1 reference

https://pubmed.ncbi.nlm.nih.gov/20194130

12 December 2020

based on heuristic

inferred from PubMed ID database lookup

Identifiers

10.1113/JPHYSIOL.2009.180224

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

PMC publication ID

0 references

PubMed publication ID

1 reference

Europe PubMed Central

PubMed publication ID

4 November 2017

ResearchGate publication ID

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