PMID: 18281290

Babenko AP
A novel ABCC8 (SUR1)-dependent mechanism of metabolism-excitation uncoupling.
J Biol Chem. 2008 Apr 4;283(14):8778-82. Epub 2008 Feb 15., [PubMed]
Sentences
No. Mutations Sentence Comment
67 ABCC8 p.Gln1178Arg
X
ABCC8 p.Gln1178Arg 18281290:67:114
status: NEW
view ABCC8 p.Gln1178Arg details
 The more significant (p Ͻ 0.005) effect of the mutation at these higher MgATP/ADP ratios indicates that the Q1178R markedly amplifies the stimulatory action of [MgATP] Ͼ 0.5 mM (see later). Login to comment 80 ABCC8 p.Lys719Arg
X
ABCC8 p.Lys719Arg 18281290:80:8
status: NEW
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ABCC8 p.Lys1384Arg
X
ABCC8 p.Lys1384Arg 18281290:80:19
status: NEW
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 Second, K719R plus K1384R in the Walker A motifs eliminated the differences between the two channel activities on-cell and in 1 mM MgATP; the fractions of the Pomax were 0.003 Ϯ 0.0012 and 0.0034 Ϯ 0.0018 versus 0.0025 Ϯ 0.001 and 0.0028 Ϯ 0.0011 for NDSUR1K719RϩK1384R versus SUR1K719RϩK1384R channel, respectively (n ϭ 3 for each). Login to comment 84 ABCC8 p.Gln1178Arg
X
ABCC8 p.Gln1178Arg 18281290:84:71
status: NEW
view ABCC8 p.Gln1178Arg details
 Consistent with the latter report and my model, I hypothesize that the Q1178R mutation reduces the ability of the sulfonylurea-binding TM domain to destabilize the magnesium⅐nucleotide-bound state of the receptor. Login to comment