Figure 6.

Direct Surface Imaging and Analysis of “Central Plugs” in NPC Channels

Nuclei from control and affected individual-derived fibroblasts were exposed by hypotonic treatment and prepared for FESEM imaging as in Figure 4B. Two representative flat areas of nuclear envelopes with NPCs are shown on the left and a gallery of enlarged, individual NPC images from both categories is shown below. Individual NPCs were scored as having either an open central channel (yellow arrows, O in gallery images) or a channel occupied by a recognizable particle (blue arrows, P in gallery images). A quantitative summary is shown for >1,000 NPCs from >30 nuclei, in each category, analyzed from multiple randomly chosen flat areas of 1×1 μm. Individual A1-derived nuclei are characterized by a larger fraction of NPCs containing a particle in their central channel. Bars indicate SEM. See Figure S4 for an extended analysis of nuclei from unaffected individuals and three affected individuals from both families.