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. 2018 Dec 6;3(23):e121150. doi: 10.1172/jci.insight.121150

Figure 3. Loss of LBR or GMAP-210 in achondrogenesis 1A (ACG1A) patient–derived primary cells.

Figure 3

Immunofluorescence microscopy of patient and control fibroblasts; ACG1A_1 carries the homozygous LBR mutation described in this study, ACG1A_2 and 3 bear biallelic mutations of TRIP11; CTL_1 and 3 are representative matched wild-type controls. Cells were grown on coverslips, fixed, and costained with (A) LBR- and lamin B–specific antibodies, or (B) using antibodies directed against GMAP-210 and the cis-Golgi marker GM130. Nuclear DNA was stained with Hoechst 33342 dye. White lines indicate where red, green, and blue (RGB) fluorescence was quantified; RGB profile plots of the sections are shown on the right. Scale bars: 10 μm.