(Q24299501)

7

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author name string

J Michael Younger

1

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Liling Chen

2

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Hong-Yu Ren

3

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Meredith F N Rosser

4

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Emma L Turnbull

5

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Chun-Yang Fan

6

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Douglas M Cyr

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

8

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language of work or name

English

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publication date

11 August 2006

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published in

Cell

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volume

126

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issue

3

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page(s)

571-82

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cites work

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

Degradation of CFTR by the ubiquitin-proteasome pathway

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Polytopic proteins: preventing aggregation in the membrane

1 reference

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7 January 2021

Gp78, a membrane-anchored ubiquitin ligase, associates with Insig-1 and couples sterol-regulated ubiquitination to degradation of HMG CoA reductase

1 reference

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7 January 2021

Association of domains within the cystic fibrosis transmembrane conductance regulator

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

1 reference

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Cystic fibrosis problem probed by proteolysis

1 reference

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7 January 2021

Limited proteolysis as a probe for arrested conformational maturation of delta F508 CFTR

1 reference

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7 January 2021

Arrest of CFTRDeltaF508 folding

1 reference

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7 January 2021

Side chain and backbone contributions of Phe508 to CFTR folding

1 reference

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7 January 2021

The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.

1 reference

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7 January 2021

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

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Cotranslational ubiquitination of cystic fibrosis transmembrane conductance regulator in vitro

1 reference

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7 January 2021

Efficient intracellular processing of the endogenous cystic fibrosis transmembrane conductance regulator in epithelial cell lines

1 reference

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7 January 2021

The DeltaF508 mutation disrupts packing of the transmembrane segments of the cystic fibrosis transmembrane conductance regulator

1 reference

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7 January 2021

Assembly of ER-associated protein degradation in vitro: dependence on cytosol, calnexin, and ATP.

1 reference

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7 January 2021

Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes

1 reference

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7 January 2021

YidC family members are involved in the membrane insertion, lateral integration, folding, and assembly of membrane proteins

1 reference

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7 January 2021

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

1 reference

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7 January 2021

Derlin-2 and Derlin-3 are regulated by the mammalian unfolded protein response and are required for ER-associated degradation

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

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

1 reference

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

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

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7 January 2021

RETRACTED: Structure of MsbA from E. coli: a homolog of the multidrug resistance ATP binding cassette (ABC) transporters

1 reference

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7 January 2021

RNF5, a RING finger protein that regulates cell motility by targeting paxillin ubiquitination and altered localization

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https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

1 reference

7 January 2021

Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules

1 reference

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7 January 2021

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

1 reference

https://api.crossref.org/works/10.1016%2FJ.CELL.2006.06.041

7 January 2021

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

1 reference

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7 January 2021

Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein

1 reference

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7 January 2021