ABCMdb

PMID: 8599650

Carson MR, Welsh MJ
Structural and functional similarities between the nucleotide-binding domains of CFTR and GTP-binding proteins.
Biophys J. 1995 Dec;69(6):2443-8., [PubMed]

Sentences

No. Mutations Sentence Comment

27 ABCC7 p.Gln552His ABCC7 p.Gln552Ala ABCC7 p.His1350Gln ABCC7 p.His1350Ala To test these hypotheses we used the excised inside-out patch-clamp technique to study CFTR variants containing the Q552A, Q552H, H1350Q, and H1350A mutations. Login to comment 72 ABCC7 p.Gln552His ABCC7 p.Gln552Ala That is, Q552A and Q552H decreased the rate at A. I I 40 80 120 V (mV) I (pA) 0.50- P0 FIGURE 2 Effect of Q552 and H1350 mutations on CFTR Cl- chan- nels. Login to comment 77 ABCC7 p.Gln552His ABCC7 p.Gln552Ala ABCC7 p.His1350Gln ABCC7 p.His1350Ala Open circles, wild-type; open triangles, Q552A; open squares, Q552H; filled circles, H1350A; filled triangles, H1350Q. Login to comment 90 ABCC7 p.Gln552Ala ABCC7 p.His1350Gln ABCC7 p.His1350Ala Fig. 2 A shows 0.250- B. 2000- mean closed-time between 1000- bursts (ms) 0- C. mean burst duration (ms) wild-type T T k" IffilI -.. wild- Q552A 0552H H1350Q H1350A type 3001 wilt'- type FIGURE 3 Effect of mutation of Q552 and H1350 on single channel activity. Login to comment 113 ABCC7 p.His1350Ala Fig. 4 shows that ADP inhibited ATP-supported current in the A. H1350A NBD2 mutants to an extent similar to that observed with wild-type CFTR. Login to comment 124 ABCC7 p.His1350Gln ABCC7 p.His1350Ala A, Time course of macroscopic current in excised membrane patches from cells expressing either H1350A (top panel) or H1350Q (bottom panel) channels. Login to comment 129 ABCC7 p.His1350Gln ABCC7 p.His1350Ala There was no difference between groups (p > 0.3 for all, n = 4, 3, and 3 for wild-type, H1350A, and H1350Q, respectively). Login to comment 130 ABCC7 p.His1350Gln 301 ATP 1 ADP (pA) 1 D0 N 0 1.5 3.0 4.5 6.0 7.5 time (min) H1350Q 1 ATP 4; A 1 ADP 30- A. Login to comment 131 ABCC7 p.His1350Gln ABCC7 p.His1350Ala 'Wo I (pA) 150 0 1.5 3.0 4.5 6.0 7.5 time (min) 75- 50- % Inhibition by ADP 250- wild- H1350A H1350Q type Carson and Welsh Similarity between CFTR and GTP-Binding Proteins 2447 from the nucleotide-binding site. Login to comment 146 ABCC7 p.His1350Gln First, NBD2 mutations at K1250 that are predicted to inhibit hydrolysis prolong the duration of bursts (in contrast to the H1350Q mutation, which shortens bursts) (Carson et al., 1995). Login to comment