Figure S3.
Panel A contains two histograms comparing control and patient samples. The x-axis represents OPP-AF647 intensity, and the y-axis represents the number of cells. The histograms show three conditions: OPP 20 micromolar, OPP 20 micromolar plus CHX, and AF647. The control sample shows peaks with mean fluorescence intensities (MFI) of 2321, 272, and 72, while the patient sample shows peaks with MFIs of 2284, 516, and 61. Panel B contains two line graphs showing polysome profiles for control and patient samples. The x-axis represents the sucrose gradient, and the y-axis represents the absorbance at 254 nanometers. Both graphs show peaks corresponding to 40S, 60S, and 80S ribosomal subunits and polysomes. The control sample shows distinct peaks for 40S, 60S, and 80S subunits and a polysome region, while the patient sample shows similar peaks but with slight variations in the polysome region.
Patient-derived LCL presents with normal protein synthesis and polysome profile. (A) LCLs derived from the patient and controls were untreated or treated with CHX 100 µg/ml for 2 h, stained with OPP 20 µM for 30 min, and then analyzed by flow cytometry. Representative image of n = 3 independent experiments. (B) Cytoplasmic extracts from patient- and control-derived LCLs were separated on sucrose gradients in order to analyze their contents in ribosomal subunits and polysome fractions; a representative image of n = 2 independent experiments.