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PMID: 10099692
Seino S
ATP-sensitive potassium channels: a model of heteromultimeric potassium channel/receptor assemblies.
Annu Rev Physiol. 1999;61:337-62.,
[PubMed]
Sentences
No.
Mutations
Sentence
Comment
204
ABCC8 p.Lys719Ala
X
ABCC8 p.Lys719Ala 10099692:204:71
status:
NEW
view ABCC8 p.Lys719Ala details
ABCC8 p.Lys1384Met
X
ABCC8 p.Lys1384Met 10099692:204:119
status:
NEW
view ABCC8 p.Lys1384Met details
However, mutations of lysine residues in the Walker A motifs in NBF-1 (
K719A
) and/or the equivalent mutation in NBF-2 (
K1384M
) of SUR1 do not prevent channel inhibition by ATP (36).
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205
ABCC1 p.Gln834His
X
ABCC1 p.Gln834His 10099692:205:109
status:
NEW
view ABCC1 p.Gln834His details
Mutations in the linker region between the Walker A motif and the Walker B motif of NBF-1 (G827D, G827R, and
Q834H
), the equivalent mutations in NBF-2 (G1479D, G1479R, G1485D, G1485R, Q1486H), or the mutation of Walker B motif of NBF-2 (D1506A) do not abolish channel inhibition by ATP (105).
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207
ABCC8 p.Lys719Ala
X
ABCC8 p.Lys719Ala 10099692:207:38
status:
NEW
view ABCC8 p.Lys719Ala details
ABCC8 p.Lys1384Met
X
ABCC8 p.Lys1384Met 10099692:207:73
status:
NEW
view ABCC8 p.Lys1384Met details
In contrast, neither the NBF-1 mutant
K719A
channel nor the NBF-2 mutant
K1384M
channel is activated by MgADP (36).
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210
ABCC8 p.Lys719Ala
X
ABCC8 p.Lys719Ala 10099692:210:43
status:
NEW
view ABCC8 p.Lys719Ala details
ABCC8 p.Lys1384Met
X
ABCC8 p.Lys1384Met 10099692:210:140
status:
NEW
view ABCC8 p.Lys1384Met details
A mutation in the Walker A motif in NBF-1 (
K719A
) abolishes channel activation by diazoxide, but a mutation in the Walker A motif in NBF-2 (
K1384M
) does not affect the channel activation (36).
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211
ABCC1 p.Gln834His
X
ABCC1 p.Gln834His 10099692:211:220
status:
NEW
view ABCC1 p.Gln834His details
Mutations in the linker region and the Walker B motif of NBF-2 (G1479D, G1479R, G1485D, G1485R, Q1486H, and D1506A) abolish channel activation by diazoxide, and mutations in the linker region of NBF-1 (G827D, G827R, and
Q834H
) alter the kinetics of diazoxide activation (106).
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230
ABCC8 p.Asp854Asn
X
ABCC8 p.Asp854Asn 10099692:230:125
status:
NEW
view ABCC8 p.Asp854Asn details
ABCC8 p.Lys719Arg
X
ABCC8 p.Lys719Arg 10099692:230:52
status:
NEW
view ABCC8 p.Lys719Arg details
ABCC8 p.Lys719Met
X
ABCC8 p.Lys719Met 10099692:230:59
status:
NEW
view ABCC8 p.Lys719Met details
Both mutations of the lysine in the Walker A motif (
K719R
,
K719M
) and a mutation of the aspartic acid in the Walker B motif (
D854N
) of SUR1 impair Mg2+ -independent high-affinity ATP binding (124).
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