ABCMdb

ABCC7 p.Pro99Ala

None has been submitted yet.

Publications

PMID: 15504721 [PubMed] Ge N et al: "Direct comparison of the functional roles played by different transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore."

No. Sentence Comment

76 However, the unitary conductance was drastically reduced by some mutations in TM1 (K95Q, Q98A, P99A) and TM6 (R334K, F337A) (Figs. 2-4). Login to comment
82 In most cases macroscopic I-V relationships were linear or weakly inwardly rectifying in the presence of symmetrical high Cl- concentra- tions (as quantified in Fig. 6), although particularly strong inward rectification was observed in Q98A, P99A, and R334K. Login to comment

PMID: 9922375 [PubMed] Sheppard DN et al: "Structure and function of the CFTR chloride channel."

No. Sentence Comment

124 When the basic arginine at this position was re- gle-channel Cl0 conductance in the rank order: wild type ' P99G ú P99L ' P99A (119). Login to comment

PMID: 8663008 [PubMed] Sheppard DN et al: "Contribution of proline residues in the membrane-spanning domains of cystic fibrosis transmembrane conductance regulator to chloride channel function."

No. Sentence Comment

9 Mutant channels were regulated like wild-type CFTR; however, single-channel conductance was decreased in the rank order: wild-type CFTR P99G > P99L P99A. Login to comment
77 As an example, Fig. 2 shows data from studies of P99A, P99G, and P99L; qualitatively similar results were obtained with the Pro205 , Pro324 , and Pro1021 mutants (data not shown). Login to comment
119 Reversal potentials (Erev) of baseline subtracted cAMP-activated whole cell currents were P99A, -30 Ϯ 1 mV (n ϭ 7); P99G, -34 Ϯ 2 mV (n ϭ 5); and P99L, -24 Ϯ 3 mV (n ϭ 6). Login to comment
122 Mutant n Px/PCl Gx/GCl Br- Cl- IBr- ClI- CFTR 5 1.18 Ϯ 0.08 1.00 0.73 Ϯ 0.05 1.27 Ϯ 0.16 1.00 0.61 Ϯ 0.08 P99A 7 0.98 Ϯ 0.03 1.00 0.70 Ϯ 0.06 1.04 Ϯ 0.05 1.00 0.72 Ϯ 0.05 P99G 5 1.06 Ϯ 0.02 1.00 0.75 Ϯ 0.08 1.04 Ϯ 0.07 1.00 0.66 Ϯ 0.05 P99L 5 1.21 Ϯ 0.07 1.00 1.06 Ϯ 0.07 1.33 Ϯ 0.11 1.00 0.95 Ϯ 0.08 P205A 4 1.09 Ϯ 0.07 1.00 0.64 Ϯ 0.09 0.95 Ϯ 0.04 1.00 0.46 Ϯ 0.11 P205G 5 1.09 Ϯ 0.05 1.00 0.45 Ϯ 0.05 1.05 Ϯ 0.03 1.00 0.44 Ϯ 0.06 P205S 2 1.01 Ϯ 0.01 1.00 0.55 Ϯ 0.28 1.09 Ϯ 0.09 1.00 0.59 Ϯ 0.08 P324A 7 1.08 Ϯ 0.04 1.00 0.72 Ϯ 0.06 1.15 Ϯ 0.07 1.00 0.60 Ϯ 0.08 P324G 6 1.12 Ϯ 0.07 1.00 0.69 Ϯ 0.04 1.22 Ϯ 0.14 1.00 0.57 Ϯ 0.04 P1021A 3 1.15 Ϯ 0.17 1.00 0.73 Ϯ 0.11 1.17 Ϯ 0.10 1.00 0.47 Ϯ 0.19 P1021G 7 1.17 Ϯ 0.06 1.00 0.78 Ϯ 0.02 1.21 Ϯ 0.08 1.00 0.59 Ϯ 0.06 though for P99G the reduction was small, for P99A and P99L the effect was marked. Login to comment
126 The conductance for P99G was 7.31 Ϯ 0.24 pS (n ϭ 5), not significantly different from wild type (p ϭ 0.26). Login to comment
127 In contrast, the conductances of P99A and P99L were significantly decreased at 4.66 Ϯ 0.25 pS (n ϭ 5, p Ͻ 0.0001) and 4.97 Ϯ 0.24 pS (n ϭ 5, p Ͻ 0.0001), respectively. Login to comment
129 Because the single-channel current amplitudes of P99A and P99L were much reduced and because in most cases the patches of membrane contained large numbers of channels, we could not accurately measure single-channel kinetics. Login to comment
145 The number of cells responding to cAMP agonists with Cl- current activation relative to the total number of cells tested for each construct was: CFTR (8/16; 50%), P99A (11/12; 92%), P99G (9/19; 47%), P99L (10/19; 53%), P205A (7/12; 58%), P205G (5/9; 56%), P205S (7/20; 35%), P324A (9/18; 50%), P324G (9/22; 41%), P1021A (8/18; 44%), and P1021G (7/16; 44%). Login to comment
172 A, regulation of P99A by phosphorylation with the catalytic subunit of cAMP-dependent protein kinase (75 nM) and intracellular MgATP (0.88 mM). Login to comment
173 Representative recording are from an excised inside-out membrane patch from a HeLa cell transiently expressing P99A. Login to comment
177 B, representative single-channel recordings are from excised inside-out membrane patches from HeLa cells transiently expressing wild-type CFTR, P99A, P99G, and P99L. Login to comment
180 C, single-channel I-V relationships of CFTR (circles), P99A (squares), P99G (triangles), and P99L (inverted triangles. Login to comment
199 Substitution of alanine, glycine, and leucine at Pro99 decreased single-channel conductance in the rank order: wild-type CFTR Ն P99G Ͼ P99L Ն P99A. Login to comment
206 Implications for Cystic Fibrosis-P99L and P205S are CF mutations located in MSD1 that are associated with a milder (pancreatic sufficiency) clinical phenotype (12, 13). Login to comment
78 As an example, Fig. 2 shows data from studies of P99A, P99G, and P99L; qualitatively similar results were obtained with the Pro205 , Pro324 , and Pro1021 mutants (data not shown). Login to comment
123 Reversal potentials (Erev) of baseline subtracted cAMP-activated whole cell currents were P99A, 230 6 1 mV (n 5 7); P99G, 234 6 2 mV (n 5 5); and P99L, 224 6 3 mV (n 5 6). Login to comment
131 In contrast, the conductances of P99A and P99L were significantly decreased at 4.66 6 0.25 pS (n 5 5, p , 0.0001) and 4.97 6 0.24 pS (n 5 5, p , 0.0001), respectively. Login to comment
133 Because the single-channel current amplitudes of P99A and P99L were much reduced and because in most cases the patches of membrane contained large numbers of channels, we could not accurately measure single-channel kinetics. Login to comment
149 The number of cells responding to cAMP agonists with Cl2 current activation relative to the total number of cells tested for each construct was: CFTR (8/16; 50%), P99A (11/12; 92%), P99G (9/19; 47%), P99L (10/19; 53%), P205A (7/12; 58%), P205G (5/9; 56%), P205S (7/20; 35%), P324A (9/18; 50%), P324G (9/22; 41%), P1021A (8/18; 44%), and P1021G (7/16; 44%). Login to comment
178 Representative recording are from an excised inside-out membrane patch from a HeLa cell transiently expressing P99A. Login to comment
183 B, representative single-channel recordings are from excised inside-out membrane patches from HeLa cells transiently expressing wild-type CFTR, P99A, P99G, and P99L. Login to comment
186 C, single-channel I-V relationships of CFTR (circles), P99A (squares), P99G (triangles), and P99L (inverted triangles. Login to comment