ABCC7 p.Glu621Gly

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Publications
PMID: 20435887 [PubMed] Billet A et al: "C terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation."
No. Sentence Comment
2 We mutated CFTR amino acids located in the betac5-betac6 hairpin, within the betac5 strand (H620Q), within the beta-turn linking the two beta strands (E621G, G622D), as well as within (S623A, S624A) and at the extremity (G628R) of the betac6 strand.
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ABCC7 p.Glu621Gly 20435887:2:151
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3 Functional analysis reveals that the current density was largely reduced for G622D and G628R channels compared with wt CFTR, similar for E621G and S624A, but increased for H620Q and S623A.
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ABCC7 p.Glu621Gly 20435887:3:137
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5 In addition, in presence of the CFTR activator benzo[c]quinolizinium, the CFTR current density compared with that of wt CFTR was abolished for G622D and G628R channels, but similar for H620Q, S623A, and S624A or slightly increased for E621G.
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ABCC7 p.Glu621Gly 20435887:5:235
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33 We have mutated these two glycine residues in aspartic acid (G622D) and arginine (G628R) and considered other mutants in their neighborhood (H620Q, E621G, S623A, and S624A).
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ABCC7 p.Glu621Gly 20435887:33:148
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87 RESULTS Expression of the NBD1 C-terminal CFTR Mutants-We have introduced EGFP-tagged CFTR proteins into HEK293 cells, wt CFTR and six CFTR mutants, i.e. H620Q, E621G, G622D, S623A, S624A, and G628R.
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ABCC7 p.Glu621Gly 20435887:87:161
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90 For the expression of CFTR mutants H620Q, E621G, S623A, and S624A, the profiles of core-glycosylated and mature-glycosylated forms were similar to that of the wt protein.
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ABCC7 p.Glu621Gly 20435887:90:42
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108 First, Cl- currents recorded for wt CFTR, E621G, and S624A CFTR mutants were not significantly different (mean current densities for each construct are reported in supplemental Table 2).
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ABCC7 p.Glu621Gly 20435887:108:42
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131 On the contrary, a significant increase of I/V slope (Fig. 7A) in presence of MPB-91 was observed for E621G CFTR channels compared with wt (I/V slope of wt, 0.390 Ϯ 0.014, n ϭ 6; E621G, 0.628 Ϯ 0.018, n ϭ 8).
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ABCC7 p.Glu621Gly 20435887:131:102
status: NEW
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ABCC7 p.Glu621Gly 20435887:131:191
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173 Indeed, our recordings of Cl currents supported by CFTR mutants E621G and S624A were not different from the non-mutated channels stimulated either by Fsk or by MPB agents.
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ABCC7 p.Glu621Gly 20435887:173:64
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180 In addition, MPB-91 was also able to activate H620Q, E621G, S623A, and S624A mutants.
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ABCC7 p.Glu621Gly 20435887:180:53
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181 The Cl- current densities elicited by these mutants, except for E621G, were similar to wt.
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ABCC7 p.Glu621Gly 20435887:181:64
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190 For the CFTR E621G mutant, Cl- current activated by MPB-91 was increased compared with wt.
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ABCC7 p.Glu621Gly 20435887:190:13
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202 Thus, one might hypothesize that the E621G mutation may reinforce the effect of MPB-91 by acting on critical features of the NBD interface, for example by contributing to reinforce it.
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ABCC7 p.Glu621Gly 20435887:202:37
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