Chaperoning the maturation of the cystic fibrosis transmembrane conductance regulator

American Journal of Physiology - Lung Cellular and Molecular Physiology

Volumn 281, Issue 1 25-1, 2001, Pages

  Brodsky, Jeffrey L ^a  

^a UNIVERSITY OF PITTSBURGH   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

CHAPERONE; CYCLOPHILIN; HEAT SHOCK PROTEIN 70; PHENYLALANINE; PROTEIN DISULFIDE ISOMERASE; TRANSMEMBRANE CONDUCTANCE REGULATOR;

CELL MEMBRANE; CYSTIC FIBROSIS; ENDOPLASMIC RETICULUM; GOLGI COMPLEX; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN BINDING; PROTEIN CONFORMATION; PROTEIN DOMAIN; PROTEIN FOLDING; PROTEIN SECRETION; PROTEIN STABILITY; SHORT SURVEY;

EID: 0034813890     PISSN: 10400605     EISSN: None     Source Type: Journal    
DOI: 10.1152/ajplung.2001.281.1.l39     Document Type: Short Survey

References (40)

1. Upf1 and Upf2 proteins mediate normal yeast mRNA degradation when translation initiation is limited
2. Ubiquitin-dependent degradation of certain protein substrates in vitro requires the molecular chaperone Hsc70
3. Affinity panning of a library of peptides displayed on bacteriophages reveals the binding specificity of BiP
4. ER protein quality control and proteasome-mediated protein degradation
5. Chemical chaperones correct the mutant phenotype of the delta F508 cystic fibrosis transmembrane conductance regulator protein
6. Chemical chaperones mediate increased secretion of mutant α1 antitrypsin (α1-AT) Z: A potential pharmacological strategy for prevention of liver injury and emphysema in α1-AT deficiency
7. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
8. Induction of HSP70 promotes ΔF508 CFTR trafficking
9. Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation
10. Processing of mutant cystic fibrosis transmembrane conductance regulator is temperature-sensitive
11. Chloride conductance expressed by delta F508 and other mutant CFTRs in Xenopus oocytes
12. Partial restoration of defective chloride conductance in ΔF508 CF mice by the chemical chaperone trimethylamine oxide
13. Peptide binding specificity of the molecular chaperone BiP
14. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
15. Partial restoration of cAMP-stimulated CFTR chloride channel activity in ΔF508 cells by deoxyspergualin
16. ΔF508 CFTR protein expression in tissue from patients with cystic fibrosis
17. Successive action of dnaK, dnaJ and GroEL along the pathway of chaperone-mediated folding
18. Perturbation of Hsp90 interaction with nascent CFTR prevents its maturation and accelerates its degradation by the preteasome
19. The delta F508 mutation decreases the stability of cystic fibrosis transmembrane conductance regulator in the plasma membrane. Determination of functional half-lives on transfected cells
20. Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP
21. The role of ATP in the functional cycle of the DnaK chaperone system
22. The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis
23. The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation
24. Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator
25. Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding
26. Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway
27. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
28. In vitro pharmacologic restoration of CFTR-mediated chloride transport with sodium 4-phenylbutyrate in cystic fibrosis epithelial cells containing delta F508-CFTR
29. Sodium 4-phenylbutyrate downregulates HSC70 expression by facilitating mRNA degradation
30. Sodium 4-phenylbutyrate downregulates HSC70: Implications for intracellular trafficking of ΔF508-CFTR
31. Substrate specificity of the DnaK chaperone determined by scanning cellulose-bound peptide libraries
32. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
33. Kinetics of molecular chaperone action
34. The molecular chaperone Hsc70 assists the in vitro folding of the N-terminal nucleotide-binding domain of the cystic fibrosis transmembrane conductance regulator
35. Defective protein folding as a basis of human disease
36. Degradation of CFTR by the ubiquitin-proteasome pathway
37. Function of DnaJ and DnaK as chaperones in origin-specific DNA binding by RepA
38. Evidence that endoplasmic reticulum (ER)-associated degradation of cystic fibrosis transmembrane conductance regulator is linked to retrograde translocation from the ER membrane
39. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
40. Limited proteolysis as a probe for arrested conformational maturation of ΔF508 CFTR