ABCC7 p.Glu726Lys
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PMID: 12361483
[PubMed]
Chen EY et al: "The PEST sequence does not contribute to the stability of the cystic fibrosis transmembrane conductance regulator."
No.
Sentence
Comment
48
The E725K/E726K mutant was included to test if alteration of charges would affect the function of the PEST sequence.
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ABCC7 p.Glu726Lys 12361483:48:10
status: NEW109 Misfolded or misassembled proteins, such as processing defective ∆F508 mutant CFTR, are recognized and retained in the ER by the quality control system in the ER; although the exact mechanism for recognition is yet to be elucidated, evidence indicate that the prolonged association with mo- Table 1: Mutations introduced into the predicted PEST sequence of CFTR Mutant Sequence PEST Score Wild-type (WT) 716 - KTPLQMNGIEEDSDEPLER - 734 +6.91 Poly-Valine 716 - KTPLQMNGIVVVVVVPLER - 734 -26.19 E725K/E726K 716 - KTPLQMNGIKKDSDEPLER - 734 N/A S728A 716 - KTPLQMNGIEEDADEPLER - 734 +4.07 T717A/E725K 716 - KAPLQMNGIKEDSDEPLER - 734 N/A * Residues in bold are the mutations introduced.
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ABCC7 p.Glu726Lys 12361483:109:506
status: NEW134 Mature Immature WT CFTR Mature Immature ∆F508 CFTR WT/∆F508 Poly-Valine S728A E725K/E726K T717A/E725K A. B.
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ABCC7 p.Glu726Lys 12361483:134:98
status: NEW135 205 130 90 kDa 100 kD 81 kD Immature ∆F508 Poly-Valine ∆F508/Poly-Valine ∆F508/S728A ∆F508/E725K/E726K ∆F508/T717A/E725K volved in protein-protein interactions: direct interaction with ubc9 [33] and ligand recognition [34,35].
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ABCC7 p.Glu726Lys 12361483:135:125
status: NEW153 A) Pulse-chase radiograph for non-processing defective constructs: WT, WT/E725K/E726K, and WT/T717A/E725K; results for WT/S728A not shown, but is similar to that of WT.
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ABCC7 p.Glu726Lys 12361483:153:80
status: NEW154 B) Pulse-chase radiograph for processing defective constructs: ∆F508, ∆F508/poly-valine, ∆F508/E725K/E726K, and ∆F508/T717/E725K; results for WT/Poly-valine and ∆F508/S728A not shown but they show similar results as ∆F508.
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ABCC7 p.Glu726Lys 12361483:154:122
status: NEW156 Hours 0 2 4 6 8 12 24 E725K/E726K Mature Immature T717A/E725KMature Immature WT Mature Immature A.
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ABCC7 p.Glu726Lys 12361483:156:28
status: NEW157 Hours 0 2 4 6 8 12 24 ∆∆∆∆F508Mature Immature ∆∆∆∆F508/ E725K/E726K Mature Immature ∆∆∆∆F508/ T717A/E725K Mature Immature Mature Immature ∆∆∆∆F508/ Poly-Valine B. mutant protein to not mature suggests otherwise.
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ABCC7 p.Glu726Lys 12361483:157:118
status: NEW177 0 0.1 1 10 100 1000 [Trypsin] (µg/ml) T717A/E725KMature Immature E725K/E726KMature Immature S728AMature Immature WTMature Immature Poly-ValineMature Immature ∆F508Mature Immature 0 0.1 1 10 100 1000 [Trypsin] (µg/ml) ∆F508/ Poly-Valine Mature Immature ∆F508/ S728A Mature Immature ∆F508/ E725K/E726K Mature Immature ∆F508/ T717A/E725K Mature Immature the PEST region of another nuclear SUMO-1 target protein, HIPK2 [48].
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ABCC7 p.Glu726Lys 12361483:177:332
status: NEW