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

Figure 5. Endoplasmic reticulum (ER) and Golgi morphology in achondrogenesis 1A (ACG1A).

Figure 5

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_3 is representative of matched wild-type controls (n = 3). Cells were grown on coverslips, fixed, and (A) costained with antibodies directed against the ER marker protein disulfide isomerase (PDI) and the trans-Golgi protein p230, or (B) using antibodies directed against the cis- and medial-Golgi protein giantin and the LBR ligand lamin B. Nuclear DNA was stained with Hoechst 33342 dye. Scale bars: 10 μm.