Figure 6
Adhesion and migration characteristics of LAD-III B cells expressing human WT and mutant kindlin-3-GFP. (A) DIC and IRM images of the LAD-III patient's EBV-transformed B cells transfected with GFP cDNA were compared with cells transfected with WT KINDLIN3, Gly308Arg, Pro425ArgAlaX, and double mutant KINDLIN3 cDNAs. Images are representative of 20 fields per sample; n = 3. Quantification of the area of close contact from one typical experiment; n = 30 cells/group. Data are shown as mean ± SEM; ***P < .001; NS indicates not significant. Scale bar represents 10 μm. (B) Migration characteristics of LAD-III B cells transfected with GFP cDNA were compared with cells transfected with WT KINDLIN3-GFP, Gly308Arg, Pro425ArgAlaX, and double mutant KINDLIN3-GFP cDNAs. n = 3 for each cDNA construct. Single-cell tracking images are accompanied by quantification of average speed of migration for n = 15 cells/group from a representative experiment. Data are shown as mean ± SEM; **P < .01. (C) Model of the kindlin-3 PH domain showing location of the Pro425AlaArgX truncation mutation (*) in the loop between the β6 and β7 strands.

Adhesion and migration characteristics of LAD-III B cells expressing human WT and mutant kindlin-3-GFP. (A) DIC and IRM images of the LAD-III patient's EBV-transformed B cells transfected with GFP cDNA were compared with cells transfected with WT KINDLIN3, Gly308Arg, Pro425ArgAlaX, and double mutant KINDLIN3 cDNAs. Images are representative of 20 fields per sample; n = 3. Quantification of the area of close contact from one typical experiment; n = 30 cells/group. Data are shown as mean ± SEM; ***P < .001; NS indicates not significant. Scale bar represents 10 μm. (B) Migration characteristics of LAD-III B cells transfected with GFP cDNA were compared with cells transfected with WT KINDLIN3-GFP, Gly308Arg, Pro425ArgAlaX, and double mutant KINDLIN3-GFP cDNAs. n = 3 for each cDNA construct. Single-cell tracking images are accompanied by quantification of average speed of migration for n = 15 cells/group from a representative experiment. Data are shown as mean ± SEM; **P < .01. (C) Model of the kindlin-3 PH domain showing location of the Pro425AlaArgX truncation mutation (*) in the loop between the β6 and β7 strands.

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