ΔF508-CFTR causes constitutive NF-κB activation through an ER-overload response in cystic fibrosis lungs

Biological Chemistry

Volumn 383, Issue 2, 2002, Pages 271-282

  Knorre, Alexander ^a   Wagner, Mathias ^a   Schaefer, Hans Eckart ^a   Colledge, William H ^a   Pahl, Heike L ^a  

^a UNIVERSITY OF FREIBURG   (Germany)

Author keywords

Endoplasmic reticulum; ER storage diseases; Transcription factors

Indexed keywords

CYTOKINE; IMMUNOGLOBULIN ENHANCER BINDING PROTEIN; TRANSCRIPTION FACTOR; TRANSMEMBRANE CONDUCTANCE REGULATOR; CFTR PROTEIN, HUMAN; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR DELTA F508;

ANIMAL TISSUE; ARTICLE; CONTROLLED STUDY; DEATH; DISEASE COURSE; ENDOPLASMIC RETICULUM; GENE MUTATION; HUMAN; HUMAN CELL; HUMAN TISSUE; INFLAMMATION; LUNG FIBROSIS; LUNG INFECTION; MOUSE; NONHUMAN; PRIORITY JOURNAL; PROTEIN EXPRESSION; PROTEIN FOLDING; RECURRENT DISEASE; RESPIRATORY FAILURE; STRESS; TRANSCRIPTION INITIATION; ANIMAL; BIOSYNTHESIS; CELL LINE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR MOUSE; DISEASE MODEL; GENETICS; LUNG; METABOLISM; TRANSACTIVATION; ULTRASTRUCTURE;

ANIMALIA;

ANIMALS; CELL LINE; CYSTIC FIBROSIS; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; DISEASE MODELS, ANIMAL; ENDOPLASMIC RETICULUM; HUMANS; LUNG; MICE; MICE, INBRED CFTR; NF-KAPPA B; TRANS-ACTIVATION (GENETICS);

EID: 0036005866     PISSN: 14316730     EISSN: None     Source Type: Journal    
DOI: 10.1515/BC.2002.029     Document Type: Article

References (60)

1. Intracellular protein trafficking defects in human disease
2. Integration of endoplasmic reticulum signaling in health and disease
3. Lower respiratory infection and inflammation in infants with newly diagnosed cystic fibrosis
4. Lower airway inflammation in infants and young children with cystic fibrosis
5. IκB: A specific inhibitor of the NF-κB transcription factor
6. Function and activation of NF-κB in the immune system
7. The relationship between infection and inflammation in the early stages of lung disease from cystic fibrosis
8. Tumor necrosis factor and interleukin-1 lead to phosphorylation and loss of IκB-α: A mechanism for NF-κB activation
9. Protease-antiprotease imbalance in the lungs of children with cystic fibrosis
10. Inflammatory cytokines in cystic fibrosis lungs
11. Hepatic endoplasmic reticulum storage diseases
12. Cystic fibrosis lung inflammation: Early, sustained, and severe
13. Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
14. Genetic basis of variable exon 9 skipping in cystic fibrosis transmembrane conductance regulator mRNA
15. Generation and characterization of a delta F508 cystic fibrosis mouse model
16. Abnormal expression of the cystic fibrosis transmembrane regulator in chronic sinusitis in cystic fibrosis and non-cystic fibrosis patients
17. CFTR expression and chloride secretion in polarized immortal human bronchial epithelial cells
18. Activation of NF-κB by adherent Pseudomonas aeruginosa in normal and cystic fibrosis respiratory epithelial cells
19. CFTR and differentiation markers expression in non-CF and delta F 508 homozygous CF nasal epithelium
20. Increased bronchial chloride concentration in cystic fibrosis
21. Rapid proteolysis of IκB-α is necessary for activation of transcription factor NF-κB
22. Cystic fibrosis of the pancreas: An estimate of the incidence
23. Tyrosine phosphorylation of IκB-α activates NF-κB without proteolytic degradation of IκB-α
24. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
25. Selective recognition of the activated form of transcription factor NF-κB by a monoclonal antibody
26. Altered cytokine production by cystic fibrosis tracheal gland serous cells
27. Identification of the cystic fibrosis gene: Genetic analysis
28. Early pulmonary inflammation in infants with cystic fibrosis
29. Bronchoalveolar lavage findings in cystic fibrosis patients with stable, clinically mild lung disease suggest ongoing infection and inflammation
30. The mechanism of Z α 1-antitrypsin accumulation in the liver
31. 2 and antioxidants have opposite effects on activation of NF-κB and AP-1 in intact cells: AP-1 as secondary antioxidant-responsive factor
32. Quantitation of inflammatory responses to bacteria in young cystic fibrosis and control patients
33. Interleukin-8 gene expression in human bronchial epithelial cells
34. Nasal and bronchoalveolar lavage fluid cytokines in early cystic fibrosis
35. Activators and target genes of Rel/NF-κB transcription factors
36. Signal transduction from the endoplasmic reticulum to the cell nucleus
37. A novel signal transduction pathway from the endoplasmic reticulum to the nucleus is mediated by transcription factor NF-κB
38. The ER-overload response - Activation of NF-κB
39. Differential localization of the cystic fibrosis transmembrane conductance regulator in normal and cystic fibrosis airway epithelium
40. Physiological basis of cystic fibrosis: A historical perspective
41. Production of a severe cystic fibrosis mutation in mice by gene targeting
42. Identification and characterization of an IκB kinase
43. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
44. Identification of the cystic fibrosis gene: Chromosome walking and jumping
45. Autoregulation of the NF-κB transactivator RelA (p65) by multiple cytoplasmic inhibitors containing ankyrin motifs
46. Liver disease in α1-antitrypsin deficiency detected by screening of 200 000 infants
47. Selective up-regulation of chemokine IL-8 expression in cystic fibrosis bronchial gland cells in vivo and in vitro
48. Genistein inhibits constitutive and inducible NFκB activation and decreases IL-8 production by human cystic fibrosis bronchial gland cells
49. High susceptibility for cystic fibrosis human airway gland cells to produce IL-8 through the IκB kinase α pathway in response to extracellular NaCl content
50. Inflammation and infection in naive human cystic fibrosis airway grafts
51. A proteasome inhibitor prevents activation of NF-κB and stabilizes a newly phosphorylated form of IκB-α that is still bound to NF-κB
52. Phosphorylation of human IκB-α on serine 32 and 36 controls IκB-α proteolysis and NF-κB activation in response to diverse stimuli
53. Management and care of the newly diagnosed patient with cystic fibrosis
54. Exaggerated activation of nuclear factor-κB and altered IκB-β processing in cystic fibrosis bronchial epithelial cells
55. Degradation of CFTR by the ubiquitin-proteasome pathway
56. Cystic fibrosis problem probed by proteolysis
57. IκB Kinase-β: NF-κB activation and complex formation with IκB kinase-α and NIK
58. The common variant of cystic fibrosis transmembrane conductance regulator is recognized by hsp70 and degraded in a pre-Golgi nonlysosomal compartment
59. Cystic fibrosis: Genotypic and phenotypic variations