Vesicles accumulating in the MPSIIIB mouse rostral cortex are distinct from lysosomes. A–M: Rostral cortex fragments of 8-month-old wild-type (A) or MPSIIIB mice (B–M) were processed for electron microscopy on ultrathin sections. Low magnification images of wild-type (A) and MPSIIIB neurons (B) show polymorphic distended vesicles in the affected cell. A normal lysosome is shown in A (inset). High magnifications of vesicles present in MPSIIIB neuron soma (C–I) show intraluminal electro-dense fibrillar (C), granular (D), globular (arrowheads in E), or multilamellar (arrows in E–G) materials. A sequestered vesicle is visible in H (double arrowhead). Images consistent with vesicle fusion or scission were observed (I). Vesicles with zebra bodies are shown in I. Enlargement of a process filled with dense vesicular bodies is visible in J. Vesicles also accumulate in myelinated axons (K) and in dendrites (L–M). A dendritic multivesicular body with normal aspect juxtaposed to a vesicle loaded with multilamellar material (arrow) is shown (M). Azure stains tissue beyond cell limits. Ly, lysosome; mye, myelin; MVB, multivesicular body; n, nucleus. Scale bars, 1 μm (A, B, J, and L); 0.2 μm (C–H, K, and M); 0.4 μm (I). N and O: Ultrathin cryosections from wild-type (N) or MPSIIIB (O) rostral cortex were immunostained for LAMP1 using 15 nm gold particles, revealing LAMP1 in small vesicles (N) or in limiting membranes of most distended vesicles (O). Scale bars, 0.2 μm.