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Science, ISSN 0036-8075, 04/2015, Volume 348, Issue 6233, pp. 444 - 448
In cells, biosynthetic machinery coordinates protein synthesis and folding to optimize efficiency and minimize off-pathway outcomes. However, it has been... 
NBD1 | NMR-SPECTROSCOPY | MULTIDISCIPLINARY SCIENCES | IN-VIVO | CYSTIC-FIBROSIS | DELTA-F508 CFTR | RIBOSOME | REVEALS | Post-translational modification | Protein research | Protein biosynthesis | Research | Protein folding | Optimization techniques | Molecules | Cells | Proteins | Cellular | Regulators | Synthesis | Cystic fibrosis | Conductance | Folding | Assembly
Journal Article
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, ISSN 1569-1993, 06/2019
Eluforsen is an antisense oligonucleotide designed to bind to the mRNA region around the F508-encoding deletion and restore the cystic fibrosis transmembrane... 
Journal Article
Chemical Science, ISSN 2041-6520, 02/2015, Volume 6, Issue 2, pp. 1237 - 1246
Many cellular functions necessary for life are tightly regulated by protein allosteric conformational change, and correlated dynamics between protein regions... 
PROTEIN STABILITY | TEMPERATURE | MUTATION | ALLOSTERY | DISCRETE MOLECULAR-DYNAMICS | NUCLEOTIDE-BINDING | CHLORIDE CHANNEL | IDENTIFICATION | RESIDUES | CHEMISTRY, MULTIDISCIPLINARY | DELTA-F508 CFTR
Journal Article
Journal Article
European Journal of Human Genetics, ISSN 1018-4813, 12/2018, Volume 26, Issue 12, pp. 1832 - 1839
The high incidence of cystic fibrosis (CF) is due to the frequency of the c.1521_1523delCTT variant in the cystic fibrosis transmembrane conductance regulator... 
CFTR MUTATIONS | DELTA-F508 | ORIGIN | GENE | BIOCHEMISTRY & MOLECULAR BIOLOGY | DISEASE | GENETICS & HEREDITY | HAPLOTYPES | ASSOCIATION | Haplotypes | Microsatellites | Family studies | Cystic fibrosis | Conductance | Age | Heterozygosity
Journal Article
SCIENTIFIC REPORTS, ISSN 2045-2322, 09/2019, Volume 9
Loss-of-function mutations in the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) channel in human airway epithelial cells are responsible for... 
TRANSMEMBRANE CONDUCTANCE REGULATOR | IN-VITRO | CHLORIDE CHANNELS | STABILITY | MEMBRANE | MULTIDISCIPLINARY SCIENCES | DISEASE | MUTATION | LUMACAFTOR-IVACAFTOR | QUALITY-CONTROL | DELTA-F508 CFTR
Journal Article
Science, ISSN 0036-8075, 8/2010, Volume 329, Issue 5993, pp. 805 - 810
Therapeutic efforts to restore biosynthetic processing of the cystic fibrosis transmembrane conductance regulator lacking the F508 residue (ΔF508CFTR) are... 
Cellular metabolism | RESEARCH ARTICLES | Internalization | Small interfering RNA | HeLa cells | Immunoblotting | Cystic fibrosis | Cell membranes | Biosynthesis | Recycling | Physiological regulation | TRANSMEMBRANE CONDUCTANCE REGULATOR | CFTR-DELTA-F508 | CELLS | CHIP | RETICULUM-ASSOCIATED DEGRADATION | MULTIDISCIPLINARY SCIENCES | ENDOPLASMIC-RETICULUM | CYSTIC-FIBROSIS | HSC70 | E3 UBIQUITIN LIGASE | SECRETORY PATHWAY | Temperature | Molecular Chaperones - metabolism | HSC70 Heat-Shock Proteins - metabolism | Humans | Homeostasis | Ubiquitination | Lysosomes - metabolism | Cell Membrane - metabolism | Protein Stability | Ubiquitinated Proteins - metabolism | Recombinant Proteins - metabolism | HSC70 Heat-Shock Proteins - genetics | Ubiquitinated Proteins - chemistry | Ubiquitin-Protein Ligases - metabolism | Mutant Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - metabolism | Glycosylation | Cystic Fibrosis Transmembrane Conductance Regulator - chemistry | Protein Folding | Protein Transport | Mutant Proteins - chemistry | HSP90 Heat-Shock Proteins - metabolism | Cystic Fibrosis Transmembrane Conductance Regulator - genetics | Protein Conformation | RNA, Small Interfering | HeLa Cells | Ubiquitin-Protein Ligases - genetics | Physiological aspects | Research | Membrane proteins | Proteins | Plasma | Membranes | Cellular biology
Journal Article
Journal Article
Journal of Cystic Fibrosis, ISSN 1569-1993, 2011, Volume 10, Issue 2, pp. S86 - S102
Journal Article
NATURE STRUCTURAL & MOLECULAR BIOLOGY, ISSN 1545-9985, 01/2005, Volume 12, Issue 1, pp. 17 - 25
Journal Article
Human Mutation, ISSN 1059-7794, 06/2019, Volume 40, Issue 6, pp. 742 - 748
Pharmacological rescue of mutant cystic fibrosis transmembrane conductance regulator (CFTR) in cystic fibrosis (CF) depends on the specific defect caused by... 
chloride channel | cystic fibrosis | CFTR | airway epithelium | RNA splicing | MECHANISM | CORRECTORS | POTENTIATOR | IN-VITRO | DELTA-F508 | RESCUE | GENETICS & HEREDITY | THERAPIES | CYSTIC-FIBROSIS | Cystic fibrosis | Genetic aspects | Codon | Analysis | Transfection | Splicing | Epithelial cells | Missense mutant | mRNA | Mutation | Drug therapy
Journal Article
Biochemical Society Transactions, ISSN 0300-5127, 10/2018, Volume 46, Issue 5, pp. 1093 - 1098
CFTR (ABCC7) is a phospho-regulated chloride channel that is found in the apical membranes of epithelial cells, is gated by ATP and the activity of the protein... 
protein structure | transmembrane proteins | cystic fibrosis transmembrane conductance regulator | ion channel | TRANSMEMBRANE CONDUCTANCE REGULATOR | PROTEIN | PHOSPHORYLATION | BIOCHEMISTRY & MOLECULAR BIOLOGY | MUTATION | ATP BINDING | ABC TRANSPORTERS | CHLORIDE CHANNEL | NUCLEOTIDE-BINDING DOMAIN | IDENTIFICATION | DELTA-F508 CFTR
Journal Article