ABCC7 p.Asp567Ala
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PMID: 18361776
[PubMed]
Loo TW et al: "Correctors promote folding of the CFTR in the endoplasmic reticulum."
No.
Sentence
Comment
49
The COPII exit motif (Y563 KDAD567 ) was disrupted by introducing the Y563N or D565A/D567A changes into wild-type CFTR [21] or into the double-cysteine mutants.
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ABCC7 p.Asp567Ala 18361776:49:85
status: NEW63 Cell-surface labelling of CFTR Wild-type CFTR or COPII exit motif mutant Y563N or D565A/ D567A was transiently expressed in HEK-293 cells.
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ABCC7 p.Asp567Ala 18361776:63:89
status: NEW145 Tyr563 , Asp565 and Asp567 in the exit motif are evolutionarily Figure 5 Effect of COPII mutations on cross-linking of cysteine mutants (A) Whole-cell SDS extracts of HEK-293 cells expressing wild-type CFTR and wild-type CFTR containing Y563N or D565A/D567A mutation were subjected to immunoblot analysis.
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ABCC7 p.Asp567Ala 18361776:145:255
status: NEW146 (B) HEK293 cells expressing wild-type CFTR or wild-type CFTR containing Y563N or D565A/D567A mutant were surface-labelled with sulfo-NHS-SS-biotin.
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ABCC7 p.Asp567Ala 18361776:146:87
status: NEW158 Accordingly, we introduced the Y563N or D565A/D567A mutations in the COPII exit motif of wild-type CFTR.
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ABCC7 p.Asp567Ala 18361776:158:46
status: NEW161 Cells expressing wild-type CFTR or Y563N or D565A/D567A mutant were treated with sulfo-NHS-SS-biotin, and then lysed with detergent.
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ABCC7 p.Asp567Ala 18361776:161:50
status: NEW
PMID: 23303252
[PubMed]
Kakoi S et al: "COPII machinery cooperates with ER-localized Hsp40 to sequester misfolded membrane proteins into ER-associated compartments."
No.
Sentence
Comment
108
Therefore we examined the ability of the diacidic EGFP-CFTR-D567A mutant to be sequestered into ERACs, but this did not significantly affect its entry into the ERAC (Figure 5).
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ABCC7 p.Asp567Ala 23303252:108:60
status: NEW113 Wild-type (ANY21) cells containing the EGFP-CFTR plasmid (top) or EGFP-CFTR-D567A plasmid (bottom) were grown to mid-log phase at 23&#ba;C and incubated with copper-containing medium for 2 h to induce expression.
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ABCC7 p.Asp567Ala 23303252:113:76
status: NEW186 The mutation to alanine at position 567 of CFTR was introduced into the gene of EGFP-CFTR in plasmid pEGFP-CFTR using primer-directed mutagenesis, yielding pEGFP-CFTR-D567A.
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ABCC7 p.Asp567Ala 23303252:186:167
status: NEW
PMID: 23890012
[PubMed]
Farinha CM et al: "Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction."
No.
Sentence
Comment
171
CFTR with Mutated Diacidic Exit Code Is Rescued by Low Temperature, but Not by Correctors (A) BHK cell lines stably expressing WT-CFTR or bearing the DAA (D567A) mutation alone or in cis with 4RK were cultured at 37 C, incubated at 26 C for 48 hr or for 24hr with VRT-325, Corr-4a, or VX-809, as indicated. CFTR protein was analyzed by WB. Data are representative of n = 5 experiments.
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ABCC7 p.Asp567Ala 23890012:171:155
status: NEW174 (C) BHK cell lines stably expressing WT-CFTR or bearing the DD/AA mutations (D565A, D567A) alone or in cis with 4RK were cultured at 37 C, incubated at 26 C for 48 hr or for 24 hr with VRT-325, Corr-4a, or VX-809, as indicated. CFTR protein was analyzed by WB. Data are representative of n = 6 experiments.
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ABCC7 p.Asp567Ala 23890012:174:84
status: NEW231 EXPERIMENTAL PROCEDURES Cells and Culture Conditions BHK cell lines expressing F508del-4RK (R29K/R516K/R555K/R716K)-, F508del-G550E-, F508del-R1070W-, F508del-V510D-, F508del-R555K-, F508del-V510D/G550E-, F508del-G550E/R1070W-, DAA (D567A)-, 4RK- DAA-, DD/AA (D565A, D567A)-, 4RK-DD/AA-, and R560T-CFTR were produced and cultured as previously described (Roxo-Rosa et al., 2006).
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ABCC7 p.Asp567Ala 23890012:231:233
status: NEWX
ABCC7 p.Asp567Ala 23890012:231:267
status: NEW