Electron microscope examination of the striatum of <i>Naglu</i> mutant mice.

<p>(<b>A</b>) Structural integrity of capillaries in the striatum was normal in C57 BL/6J control mice. (<b>B</b>) A single layer of endothelial cells surrounded by a layer of basement membrane in control mouse seen at high magnification. (<b>C</b>), (<b>D</b>) Edematous space and large vacuoles are indicated around vessels. Vacuolated pericytes can be seen in the striatum of early symptomatic <i>Naglu</i> mice. (<b>E</b>) Large vacuoles were found in endothelial cells and pericytes of late symptomatic animals. (<b>F</b>) A high magnification image of a capillary from the striatum of a 6 month old mutant mouse showing an endothelial cell with endoplasmic reticulum swelling and formation of a large vacuole in its cytoplasm. Edematous space has appeared around the capillary. Multiple layers of endothelial cells and basement membrane can be observed here, indicating a reparative process taking place. The luminal endothelial cells are damaged<b>. EC</b> - endothelial cell, <b>BM</b> - basement membrane, <b>E</b> – erythrocyte, <b>m</b> – mitochondrion, <b>A</b> – axon, <b>P</b> – pericyte, <b>PM</b> – perivascular macrophage, <b>Nu</b> – nucleus, <b>V</b> – vacuole, <b>+</b> - swollen endoplasmic reticulum, <b>asterisks</b> - microvilli, <b>></b> - extracellular edematous space. Magnifications: (<b>A</b>), (<b>C</b>): 7,100x; (<b>B</b>), (<b>E</b>): 14,000x; (<b>D</b>): 8,900x; (<b>F</b>): 28,000x.