Cystic fibrosis: premature degradation of mutant proteins as a molecular disease mechanism.

No abstract is available for this paper.

• Publication year

  2003

• Venue

  Methods in molecular biology

• Publication date

  Unknown publication date

• Fields of study

  Biology, Medicine, Chemistry

• Identifiers
  DOI 10.1385/1-59259-394-1:27PMID 12840537
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• A Principal Role for the Proteasome in Endoplasmic Reticulum-associated Degradation of Misfolded Intracellular Cystic Fibrosis Transmembrane Conductance Regulator*

  2002cited by this paper
• Calcium-pump inhibitors induce functional surface expression of ΔF508-CFTR protein in cystic fibrosis epithelial cells

  2002cited by this paper
• Cell-based assay for high-throughput quantitative screening of CFTR chloride transport agonists.

  2001cited by this paper
• Conformational and Temperature-sensitive Stability Defects of the ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator in Post-endoplasmic Reticulum Compartments*

  2001cited by this paper
• Increased functional cell surface expression of CFTR and DeltaF508-CFTR by the anthracycline doxorubicin.

  2001cited by this paper
• The ΔF508 mutation shortens the biochemical half-life of plasma membrane CFTR in polarized epithelial cells

  2001cited by this paper
• A conserved ubiquitin ligase of the nuclear envelope/endoplasmic reticulum that functions in both ER-associated and Matalpha2 repressor degradation.

  2001cited by this paper
• Correction of delF508-CFTR activity with benzo(c)quinolizinium compounds through facilitation of its processing in cystic fibrosis airway cells.

  2001cited by this paper
• Redundancy of mammalian proteasome beta subunit function during endoplasmic reticulum associated degradation.

  2001cited by this paper
• Novel CFTR Chloride Channel Activators Identified by Screening of Combinatorial Libraries Based on Flavone and Benzoquinolizinium Lead Compounds* 210

  2001cited by this paper
• Cystic fibrosis: review of the decade.

  2001cited by this paper
• Pharmacologic restoration of αδF508 CFTR-mediated chloride current

  2000cited by this paper
• Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX.

  2000cited by this paper
• Surface expression of the cystic fibrosis transmembrane conductance regulator mutant DeltaF508 is markedly upregulated by combination treatment with sodium butyrate and low temperature.

  2000cited by this paper
• Hrd1p/Der3p is a membrane-anchored ubiquitin ligase required for ER-associated degradation

  2000cited by this paper
• Pharmacological chaperones rescue cell-surface expression and function of misfolded V2 vasopressin receptor mutants.

  2000cited by this paper
• Traffic Pattern of Cystic Fibrosis Transmembrane Regulator through the Early Exocytic Pathway

  2000cited by this paper
• The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation

  2000cited by this paper
• Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy

  2000cited by this paper
• Pharmacological chaperones: a new twist on receptor folding.

  2000cited by this paper
• Evidence That Endoplasmic Reticulum (ER)-associated Degradation of Cystic Fibrosis Transmembrane Conductance Regulator Is Linked to Retrograde Translocation from the ER Membrane*

  1999cited by this paper
• ΔF508 CFTR protein expression in tissues from patients with cystic fibrosis

  1999cited by this paper
• The Hdj‐2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis

  1999cited by this paper
• Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis.

  1999cited by this paper
• Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the proteasome

  1998cited by this paper
• Aggresomes: A Cellular Response to Misfolded Proteins

  1998cited by this paper
• Cotranslational Ubiquitination of Cystic Fibrosis Transmembrane Conductance Regulator in Vitro *

  1998cited by this paper
• The Mechanism Underlying Cystic Fibrosis Transmembrane Conductance Regulator Transport from the Endoplasmic Reticulum to the Proteasome Includes Sec61β and a Cytosolic, Deglycosylated Intermediary*

  1998cited by this paper
• Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by deoxyspergualin.

  1998cited by this paper
• Der3p/Hrd1p is required for endoplasmic reticulum-associated degradation of misfolded lumenal and integral membrane proteins.

  1998cited by this paper
• Membrane Trafficking of the Cystic Fibrosis Gene Product, Cystic Fibrosis Transmembrane Conductance Regulator, Tagged with Green Fluorescent Protein in Madin-Darby Canine Kidney Cells*

  1998cited by this paper
• Direct Activation of Cystic Fibrosis Transmembrane Conductance Regulator Channels by 8-Cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-Diallyl-8-cyclohexylxanthine (DAX)

  1998cited by this paper
• Limited proteolysis as a probe for arrested conformational maturation of ΔF508 CFTR

  1998cited by this paper
• Localization and Suppression of a Kinetic Defect in Cystic Fibrosis Transmembrane Conductance Regulator Folding*

  1997cited by this paper
• Correction of Defective Protein Kinesis of Human P-glycoprotein Mutants by Substrates and Modulators*

  1997cited by this paper
• FLAG epitope positioned in an external loop preserves normal biophysical properties of CFTR.

  1997cited by this paper
• Correcting temperature-sensitive protein folding defects.

  1997cited by this paper
• Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis Mutation (*)

  1996cited by this paper
• Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein.

  1996cited by this paper
• Multiple proteolytic systems, including the proteasome, contribute to CFTR processing.

  1995cited by this paper
• Epitope tagging permits cell surface detection of functional CFTR.

  1995cited by this paper
• The relationship between genotype and phenotype in cystic fibrosis.

  1995cited by this paper
• Degradation of CFTR by the ubiquitin-proteasome pathway.

  1995cited by this paper
• MUTANT (DELTA F508) CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR CL- CHANNEL IS FUNCTIONAL WHEN RETAINED IN ENDOPLASMIC RETICULUM OF MAMMALIAN CELLS

  1995cited by this paper
• Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.

  1994cited by this paper
• Intracellular turnover of cystic fibrosis transmembrane conductance regulator. Inefficient processing and rapid degradation of wild-type and mutant proteins.

  1994cited by this paper
• Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP.

  1994cited by this paper
• The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment.

  1993cited by this paper
• Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium.

  1992cited by this paper
• Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive

  1992cited by this paper
• Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes.

  1991cited by this paper
• cAMP-inducible chloride conductance in mouse fibroblast lines stably expressing the human cystic fibrosis transmembrane conductance regulator.

  1991cited by this paper
• Molecular genetics of cystic fibrosis.

  1991cited by this paper
• Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis.

  1990cited by this paper
• Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA.

  1989cited by this paper

• Degradation

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• Targeting a genetic defect: cystic fibrosis transmembrane conductance regulator modulators in cystic fibrosis

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• Der1 promotes movement of misfolded proteins through the endoplasmic reticulum membrane

  2013cites this paper
• La protéine CFTR : Implication et cible thérapeutique dans la maladie osseuse chez les patients atteints de mucoviscidose.

  2012cites this paper
• Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment.

  2011cites this paper
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  2008cites this paper
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  2007cites this paper
• Altered Biogenesis of ΔF508-CFTR Following Treatment with Doxorubicin

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• Human disorders of ubiquitination and proteasomal degradation.

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