Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops.
about
Mechanisms of CFTR Folding at the Endoplasmic ReticulumPotentiation of ΔF508- and G551D-CFTR-Mediated Cl- Current by Novel HydroxypyrazolinesThe silent codon change I507-ATC->ATT contributes to the severity of the ΔF508 CFTR channel dysfunctionImpact of the F508del mutation on ovine CFTR, a Cl- channel with enhanced conductance and ATP-dependent gating.Cystic fibrosis transmembrane conductance regulator (CFTR) potentiators protect G551D but not ΔF508 CFTR from thermal instability.Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis.Generation of Distal Airway Epithelium from Multipotent Human Foregut Stem Cells.Thermal instability of ΔF508 cystic fibrosis transmembrane conductance regulator (CFTR) channel function: protection by single suppressor mutations and inhibiting channel activityDeletion of Phenylalanine 508 in the First Nucleotide-binding Domain of the Cystic Fibrosis Transmembrane Conductance Regulator Increases Conformational Exchange and Inhibits Dimerization.A synonymous codon change alters the drug sensitivity of ΔF508 cystic fibrosis transmembrane conductance regulator.Correctors of ΔF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein.Nonequilibrium gating of CFTR on an equilibrium theme.Analysis of cystic fibrosis-associated P67L CFTR illustrates barriers to personalized therapeutics for orphan diseasesCatalyst-like modulation of transition states for CFTR channel opening and closing: new stimulation strategy exploits nonequilibrium gating.Development of Automated Patch Clamp Technique to Investigate CFTR Chloride Channel Function.Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation.Phenylhydrazones as Correctors of a Mutant Cystic Fibrosis Transmembrane Conductance Regulator.CFTR potentiators partially restore channel function to A561E-CFTR, a cystic fibrosis mutant with a similar mechanism of dysfunction as F508del-CFTRRevertant mutants modify, but do not rescue, the gating defect of the cystic fibrosis mutant G551D-CFTR.Allosteric modulation balances thermodynamic stability and restores function of ΔF508 CFTR.Chaperones rescue the energetic landscape of mutant CFTR at single molecule and in cell.Partial rescue of F508del-CFTR channel gating with modest improvement of protein processing, but not stability by a dual-acting small molecule.Substitution of Yor1p NBD1 residues improves the thermal stability of Human Cystic Fibrosis Transmembrane Conductance Regulator.
P2860
Q21129277-A0F67B23-8C3C-472C-8390-A213CA14DF6EQ28550065-8753540E-930B-4563-AF96-A79B00FEBBAFQ28658805-65230C65-AE2C-45D2-AB5C-9AAF9EF16B38Q30372632-2F11EFD2-6B09-4609-9A44-2753F694A4E0Q34155612-F3566618-48D1-4F15-AAE0-17819AF7171EQ34748891-7FD17D71-1DDD-4035-85B9-CD9258F8567EQ35844405-2BAB7CAB-A43A-4548-A8DE-64807D5FCEB2Q36108986-5A6A83B1-898A-4DB7-B52A-42D47A42A129Q36281743-A25C7172-C5B8-4B5A-B681-ED3131023E0DQ36385376-164C3719-80F1-4B06-A1B9-EA94B06530A8Q36534393-D5D6101E-3DB6-45C8-A8D8-12D02A301863Q37061709-B2BAD608-3934-498C-9EB9-F5CF0DC7F21FQ37269585-17ED282E-CFE7-4497-8881-83BBEB07E582Q37727045-FD97B018-BEDF-4F4D-9A43-458E35C7C664Q37740542-7353FB1F-4134-46B3-8E31-34A53440E920Q38721354-A90F24CB-D2B0-44F4-853F-D824382703CCQ38809280-B1533276-5E45-42BB-BE0E-BC371E677A5DQ38987933-FD65129F-46C1-4651-B34C-F81D19C34C51Q39018857-7A3A35CD-1978-4030-9528-7A5A661FEDA5Q39381996-8E1FD040-3604-40D4-9C0B-64A54D743037Q41565850-A74A0D16-D9BC-4F85-B578-B2DFFBA90047Q47214928-3584FC2F-BB1C-4F7B-BB21-99B08A50A702Q47623778-29A54E00-4B8F-427F-836B-EA40CFFE5964
P2860
Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508): "rescue" by suppressor mutations in nucleotide binding domain 1 and by constitutive mutations in the cytosolic loops.
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
Thermally unstable gating of t ...... ations in the cytosolic loops.
@ast
Thermally unstable gating of t ...... ations in the cytosolic loops.
@en
type
label
Thermally unstable gating of t ...... ations in the cytosolic loops.
@ast
Thermally unstable gating of t ...... ations in the cytosolic loops.
@en
prefLabel
Thermally unstable gating of t ...... ations in the cytosolic loops.
@ast
Thermally unstable gating of t ...... ations in the cytosolic loops.
@en
P2093
P2860
P356
P1476
Thermally unstable gating of t ...... ations in the cytosolic loops.
@en
P2093
George O Okeyo
Jeong S Hong
Kevin L Kirk
P2860
P304
41937-41948
P356
10.1074/JBC.M111.296061
P407
P577
2011-09-30T00:00:00Z