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. 2021 Apr 28;10(9):1901. doi: 10.3390/jcm10091901

Figure 9.

Figure 9

Ultrastructure and immuno-gold EM of aldehyde-fixed liver biopsies from MYO5B-PFIC patient #1 (a,c–f) and control (b,g). (a) Endomembrane compartments distinguished in MYO5B-PFIC sample comprise large, ribosome-decorated unstained cisternae (marked by stars), ill-defined “swollen membrane sacks” (marked by a cross: +), abundant small vesicles and lysosomes (LY). Mitochondria (M) appear normal, peroxisomes and locally occurring large lipid droplets (indicating steatosis) are not shown in this frame of apical hepatocyte area facing bile canaliculus (BC); scale bar = 1 μm. Insert shows a mitochondrion at higher magnification; scale bar = 200 nm. (b) Control biopsy with normal, well-organized Golgi stack/TGN (G). Note LY and peroxisome (PX) nearby; Scale bar = 1 μm (c) ER-marker PDI labels specifically 100–200 nm-wide, small vesicles/tubules (arrows) identifying sER; scale bar = 500 nm. (d) PDI labelling of ribosome decorated cisternae (stars), thus, rER, that display numerous 75 nm-wide buds (arrows), likely at ER-exit sites; scale bar = 500 nm. (e) Anti-TGN46 label (arrows) at cisternae (cross) and associated vesicles identifies “swollen membrane sacks” as dilated TGN; scale bar = 1 μm. (f) Rab 11 label clusters locally at, or in close vicinity of ≈150 nm-wide vesicles in the hepatocytes close to the bile canaliculus (BC); scale bar = 500 nm. (g) Normal Rab11 distribution (arrows) in control hepatocytes; scale bar = 500 nm.