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

Figure 2.

Figure 2

Ultrastructural MVID-phenotype in duodenal biopsy (bx #1) and enteroid samples (ent.) from MYO5B-PFIC patient #1. (a) Villus enterocytes from chemically fixed biopsy showing a microvillus inclusion (asterisk) and subapical clusters of aberrant, dark recycling endosomes (RE: arrows) specific for this disease [10]; N = nucleus; scale bar = 5 μm. (b) PAS-cytochemistry at EM-level detects strongly reactive compounds in aberrant RE; scale bar = 1 μm. (c) Cryo-fixed enteroid displaying MVID-specific, subapical RE clusters, as well as huge lysosomes (LY) and autophagolysosomes (ALY), but normal Golgi/TGN (arrow-heads); scale bar = 2 μm. (d) Light micrograph of cryo-fixed enteroid highlights the high vitality of those enterocytes; scale bar = 20 μm. (e) Distinct PAS-reaction of subapical RE in cryo-fixed enteroid; scale bar = 500 nm. (f–h) Electron tomographic reconstruction of 3D-architecture of subapical RE in cryo-fixed enteroids. 2D slice (f) from an electron tomographic reconstruction with red contour lining (g) and the resulting 3D model (h) of those organelles (see also Supplementary Movie M1); scale bars = 500 nm.