The role of the UPS in cystic fibrosis (Q33307871)

25 July 2017

7 references

13 March 2019

21 March 2019

22 April 2019

28 September 2019

16 November 2019

28 January 2021

19 July 2021

The role of the UPS in cystic fibrosis (English)

1 reference

proteasome endopeptidase complex

25 July 2017

6 references

13 March 2019

21 March 2019

22 April 2019

28 September 2019

16 November 2019

28 January 2021

1 reference

28 September 2019

macromolecular substance

1 reference

16 November 2019

1 reference

6 February 2021

1 reference

19 July 2021

Emma L Turnbull

1

1 reference

25 July 2017

Meredith F N Rosser

series ordinal

2

1 reference

25 July 2017

Douglas M Cyr

series ordinal

3

1 reference

25 July 2017

language of work or name

1 reference

22 November 2007

9 references

25 July 2017

13 March 2019

21 March 2019

22 April 2019

28 September 2019

16 November 2019

28 January 2021

6 February 2021

19 July 2021

9 references

25 July 2017

13 March 2019

21 March 2019

22 April 2019

28 September 2019

16 November 2019

28 January 2021

6 February 2021

19 July 2021

8 Suppl 1

1 reference

25 July 2017

page(s)

S11

1 reference

Creative Commons Attribution 2.0 Generic

25 July 2017

copyright license

8 references

13 March 2019

21 March 2019

22 April 2019

28 September 2019

16 November 2019

28 January 2021

6 February 2021

on focus list of Wikimedia project

19 July 2021

copyright status

copyrighted

0 references

ScienceSource

0 references

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Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants

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21 June 2018

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Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation

21 June 2018

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The ubiquitin-domain protein HERP forms a complex with components of the endoplasmic reticulum associated degradation pathway

21 June 2018

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Folding of CFTR is predominantly cotranslational

21 June 2018

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BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP.

21 June 2018

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Species-specific differences in mouse and human airway epithelial biology of recombinant adeno-associated virus transduction

21 June 2018

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Recruitment of the p97 ATPase and ubiquitin ligases to the site of retrotranslocation at the endoplasmic reticulum membrane

21 June 2018

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Multiprotein complexes that link dislocation, ubiquitination, and extraction of misfolded proteins from the endoplasmic reticulum membrane

21 June 2018

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Membrane-bound Ubx2 recruits Cdc48 to ubiquitin ligases and their substrates to ensure efficient ER-associated protein degradation

21 June 2018

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Regulation of the cytoplasmic quality control protein degradation pathway by BAG2

21 June 2018

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Small-molecule correctors of defective DeltaF508-CFTR cellular processing identified by high-throughput screening.

21 June 2018

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Ubiquitin and ubiquitin-like proteins as multifunctional signals

21 June 2018

1 reference

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The retrotranslocation protein Derlin-1 binds peptide:N-glycanase to the endoplasmic reticulum

21 June 2018

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A quantitative description of the activation and inhibition of CFTR by potentiators: Genistein.

21 June 2018

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The regulation of proteasome degradation by multi-ubiquitin chain binding proteins

21 June 2018

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Cystic fibrosis transmembrane conductance regulator as a model substrate to study endoplasmic reticulum protein quality control in mammalian cells

21 June 2018

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Novel, mechanism-based therapies for cystic fibrosis

21 June 2018

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21 June 2018

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Multiubiquitylation by E4 enzymes: 'one size' doesn't fit all.

21 June 2018

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Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains.

21 June 2018

1 reference

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A foldable CFTR{Delta}F508 biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase

21 June 2018

1 reference

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Thapsigargin or curcumin does not promote maturation of processing mutants of the ABC transporters, CFTR, and P-glycoprotein

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

COPII-dependent export of cystic fibrosis transmembrane conductance regulator from the ER uses a di-acidic exit code

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Murine epithelial cells: isolation and culture

21 June 2018

1 reference

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Curcumin does not stimulate cAMP-mediated chloride transport in cystic fibrosis airway epithelial cells

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Evidence against the rescue of defective DeltaF508-CFTR cellular processing by curcumin in cell culture and mouse models.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A membrane protein complex mediates retro-translocation from the ER lumen into the cytosol

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A membrane protein required for dislocation of misfolded proteins from the ER

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Cystic fibrosis transmembrane conductance regulator degradation depends on the lectins Htm1p/EDEM and the Cdc48 protein complex in yeast

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

CHIP: a link between the chaperone and proteasome systems

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Curcumin, a major constituent of turmeric, corrects cystic fibrosis defects

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Pharmacological approaches to correcting the ion transport defect in cystic fibrosis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Distinct roles for the AAA ATPases NSF and p97 in the secretory pathway

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

CHIP: a quality-control E3 ligase collaborating with molecular chaperones

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Rescuing protein conformation: prospects for pharmacological therapy in cystic fibrosis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Tiny dancers: the integrin-growth factor nexus in angiogenic signaling

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A role for mammalian Ubc6 homologues in ER-associated protein degradation.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A principal role for the proteasome in endoplasmic reticulum-associated degradation of misfolded intracellular cystic fibrosis transmembrane conductance regulator

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A new gun in town: the U box is a ubiquitin ligase domain

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Non-conventional trafficking of the cystic fibrosis transmembrane conductance regulator through the early secretory pathway

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

From the cradle to the grave: molecular chaperones that may choose between folding and degradation

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Induction of HSP70 promotes DeltaF508 CFTR trafficking

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

ClC-2 Cl- channels in human lung epithelia: activation by arachidonic acid, amidation, and acid-activated omeprazole

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Rma1, a novel type of RING finger protein conserved from Arabidopsis to human, is a membrane-bound ubiquitin ligase

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Recognition of the polyubiquitin proteolytic signal

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Aggresomes: a cellular response to misfolded proteins

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

The ubiquitin-proteasome pathway: on protein death and cell life

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

RMA1, an Arabidopsis thaliana gene whose cDNA suppresses the yeast sec15 mutation, encodes a novel protein with a RING finger motif and a membrane anchor

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Analysis of ClC-2 channels as an alternative pathway for chloride conduction in cystic fibrosis airway cells.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Structural cues involved in endoplasmic reticulum degradation of G85E and G91R mutant cystic fibrosis transmembrane conductance regulator

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Modulation of CFTR chloride channels by calyculin A and genistein

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A demonstration using mouse models that successful gene therapy for cystic fibrosis requires only partial gene correction.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Cystic fibrosis: genotypic and phenotypic variations

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Der1, a novel protein specifically required for endoplasmic reticulum degradation in yeast

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A mouse model for the delta F508 allele of cystic fibrosis.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

A mouse model for the cystic fibrosis delta F508 mutation.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Degradation of CFTR by the ubiquitin-proteasome pathway

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

cAMP-independent activation of CFTR Cl channels by the tyrosine kinase inhibitor genistein

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Genistein, a specific inhibitor of tyrosine-specific protein kinases

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Identification of the cystic fibrosis gene: chromosome walking and jumping

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

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

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Selective inhibition of endoplasmic reticulum-associated degradation rescues DeltaF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels: therapeutic implications.

21 June 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Characterization of wild-type and deltaF508 cystic fibrosis transmembrane regulator in human respiratory epithelia

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

2-(dialkylamino)-4H-1-benzopyran-4-one derivatives modify chloride conductance in CFTR expressing cells

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Protein quality control: U-box-containing E3 ubiquitin ligases join the fold

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Post-translational disruption of the delta F508 cystic fibrosis transmembrane conductance regulator (CFTR)-molecular chaperone complex with geldanamycin stabilizes delta F508 CFTR in the rabbit reticulocyte lysate

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Genistein potentiates wild-type and delta F508-CFTR channel activity

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Generation and characterization of a delta F508 cystic fibrosis mouse model.

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

The amino-terminal portion of CFTR forms a regulated Cl- channel.

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis

31 October 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=2106362

Arrest of CFTRDeltaF508 folding

31 October 2018

1 reference

12 December 2020

Expression of cystic fibrosis transmembrane conductance regulator in a model epithelium

1 reference

12 December 2020

Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia

1 reference

12 December 2020

Association of domains within the cystic fibrosis transmembrane conductance regulator

1 reference

12 December 2020

Evidence that endoplasmic reticulum (ER)-associated degradation of cystic fibrosis transmembrane conductance regulator is linked to retrograde translocation from the ER membrane

1 reference

12 December 2020

A New Gun in Town: The U Box Is a Ubiquitin Ligase Domain

1 reference

12 December 2020

10.1186/1471-2091-8-S1-S11

Identifiers

DOI

1 reference

Dimensions Publication ID

25 July 2017

1 reference

https://scigraph.springernature.com/pub.10.1186/1471-2091-8-s1-s11

9 September 2019

1 reference

25 July 2017

1 reference