ABCC7 p.Ala1067Cys
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PMID: 19339490
[PubMed]
Wellhauser L et al: "A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability."
No.
Sentence
Comment
208
It is noteworthy that Loo et al. (2008) and Serohijos et al. (2008) showed that deletion of Phe508 impairs the chemical cross-linking that normally occurs between a non-native cysteine residue incorporated into NBD1, in the proximity of Phe508 (V510C), and a non-native cysteine introduced into the fourth intracellular loop (A1067C) Fig. 5.
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ABCC7 p.Ala1067Cys 19339490:208:326
status: NEW
PMID: 19761259
[PubMed]
Loo TW et al: "Correctors enhance maturation of DeltaF508 CFTR by promoting interactions between the two halves of the molecule."
No.
Sentence
Comment
11
Coexpression of ΔF508/N-half and C-half CFTR in the presence correctors VX-325 and corr-4a, however, restored interactions between the two halves. An interaction that was restored was that between NBD1 and TMD2 as the correctors restored cross-linking of mutant ΔF508/NBD1- (V510C)/TMD2(A1067C).
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ABCC7 p.Ala1067Cys 19761259:11:299
status: NEW78 For example, mature CFTR shows cross-linking between cysteines in TM segment 6 (N-half) and TM segment 12 (C-half) or between cysteines in NBD1 [N-half (V510C)] and ICL4 [C-half (A1067C)], but the immature form of ΔF508 does not (17, 25).
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ABCC7 p.Ala1067Cys 19761259:78:179
status: NEW101 The black dot indicates the predicted location of an introduced cysteine (A1067C) in TMD2 that will directly cross-link to a cysteine introduced into NBD1 (V510C).
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ABCC7 p.Ala1067Cys 19761259:101:74
status: NEW174 To test if correctors VX-325 and corr-4a promoted NBD1-TMD2 interactions in ΔF508 CFTR, mutant ΔF508/V510C(NBD1)/A1067C- (TMD2) was expressed in the presence or absence of the correctors.
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ABCC7 p.Ala1067Cys 19761259:174:125
status: NEW182 Expression of ΔF508/V510C(NBD1)/A1067C- (TMD2) in the presence of correctors, however, yielded mature CFTR (180 kDa protein) (Figure 5, right).
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ABCC7 p.Ala1067Cys 19761259:182:38
status: NEW
PMID: 21520952
[PubMed]
Loo TW et al: "Benzbromarone stabilizes DeltaF508 CFTR at the cell surface."
No.
Sentence
Comment
50
(C) Effect of benzbromarone on cross-linking (X-link) between cysteines in TMD1 and TMD2 (M348C/T1142C) or NBD1 and TMD2 (V510C/A1067C).7 (D) Immunoblot of cells expressing CFTR TMD1þ2 in the absence (À) or presence (þ) of 0.05 mM benzbromarone.
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ABCC7 p.Ala1067Cys 21520952:50:128
status: NEW
PMID: 21594776
[PubMed]
Loo TW et al: "Repair of CFTR folding defects with correctors that function as pharmacological chaperones."
No.
Sentence
Comment
252
The following protocols describe disulfide cross-linking between cysteines introduced into NBD1 (V510C) and the fourth intracellular loop in TMD2 (A1067C) of a F508del-CFTR processing mutant expressed in the presence or the absence of correctors.
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ABCC7 p.Ala1067Cys 21594776:252:147
status: NEW263 The next day, add 0.17 mg of (V510C)/(A1067C)/ F508del-CFTR cDNA to sterile water to give a total volume of 7.65 ml.
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ABCC7 p.Ala1067Cys 21594776:263:38
status: NEW