Figure 4
Figure 4. Flow cytometric detection of intraplatelet Munc13-4 protein. Flow cytometric analysis of intraplatelet Munc13-4 expression in 4 FHL3 patients and healthy controls using whole blood samples shipped overnight (A) and in an FHL3 patient (P4) and a healthy control using samples stored at room temperature for a week (B). Dark closed histograms represent platelets from FHL3 patients, whereas light closed histograms represent platelets from healthy controls. Open histograms represent staining with isotype controls. (C) Mean fluorescence intensity (MFI) of intraplatelet Munc13-4 staining for HLH patients and healthy controls. All of the healthy controls (n = 35) were adults. Non-FHL3 (nFHL3) patients (n = 31), as defined in Figure 3, varied in age (2 days-39 years) and included 2 patients with FHL2. Age-related variations in the MFI of Munc13-4 staining were not observed. **P < .01 by the Tukey post hoc test. (D) The sensitivities of Western blot and flow cytometric analyses for detecting Munc13-4–deficient platelets were compared.

Flow cytometric detection of intraplatelet Munc13-4 protein. Flow cytometric analysis of intraplatelet Munc13-4 expression in 4 FHL3 patients and healthy controls using whole blood samples shipped overnight (A) and in an FHL3 patient (P4) and a healthy control using samples stored at room temperature for a week (B). Dark closed histograms represent platelets from FHL3 patients, whereas light closed histograms represent platelets from healthy controls. Open histograms represent staining with isotype controls. (C) Mean fluorescence intensity (MFI) of intraplatelet Munc13-4 staining for HLH patients and healthy controls. All of the healthy controls (n = 35) were adults. Non-FHL3 (nFHL3) patients (n = 31), as defined in Figure 3, varied in age (2 days-39 years) and included 2 patients with FHL2. Age-related variations in the MFI of Munc13-4 staining were not observed. **P < .01 by the Tukey post hoc test. (D) The sensitivities of Western blot and flow cytometric analyses for detecting Munc13-4–deficient platelets were compared.

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