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Figure 2


Figure 2 Semithin (A) and ultrathin (B,C) sections from left ventricular endomyocardial biopsy of the same patient of Figures 1A and 1D. In panel A, osmiophilic bodies intensely stained by Azur II are seen in the endocardium, in the subendocardial space, and in myocardium. In the subendocardial space, they are localized in the region of empty spaces seen at H and E histology sections. In the myocardial tissue, a gradient of storage material can be appreciated from the subendocardial to the inner layer. At electron microscopy (B,C) the osmiophilic bodies appear to consist of glycosphingolipids organized in membrane-bounded bodies diffusely present in the context of the endocardium (End), occupying the subendocardial space as a free storage material and inside the myocytes (Myo). Arrows indicate membrane-bounded bodies at the boundaries between a myocardiocyte and the subendocardial space, suggesting a process of release from the cell to the extracellular space. (A) Azur II original magnification x100. (B,C) Bars = 1 µm.