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. 2007 Aug 22;27(34):9009–9021. doi: 10.1523/JNEUROSCI.2329-07.2007

Figure 6.

Figure 6.

Sulfolipid storage in the CNS. A–F, Sulfolipid storage in the spinal cord. Vibratome slices (100 μm) stained with AB (A, B, D, E) and paraffin sections of tissue blocks subjected to pre-embedding incubation with AB (C, F). Genotypes are as indicated. In CGT/ASA(+/+) (A), AB-positive structures are lacking. In ASA(−/−) (B), CST/ASA(−/−) (D), and CGT/ASA(−/−) (E), the gray matter and, less markedly, also the white matter display alcianophilic material. The amount of storage material in the gray matter is increased in CST/ASA(−/−) and CGT/ASA(−/−) mice compared with the ASA(−/−) mouse. The alcianophilic structures in the gray matter correspond mainly to neuronal perikarya filled with sulfolipid inclusions (C, F). The small AB-positive profiles mainly correspond to storage in glial cells, most probably oligodendrocyte processes. Scale bars: A, B, D, E, 200 μm; C, F, 20 μm. DH, Dorsal horn; VH, ventral horn. G–J, Sulfolipid storage in the medulla oblongata. Vibratome slices are as above. Genotypes are as indicated. Scale bar, 200 μm. In G, some AB staining is present in the reticular formation (Rt) and gracile nucleus (Gr) (although hardly recognizable at this low magnification), whereas the hypoglossal nucleus (12N) is unstained. CC, Central canal. H–J show AB-positive material in 12N and increased staining in Rt and Gr. Some regions, however, remain mostly unstained such as the dorsal vagal nucleus (10N), nucleus of solitary tract (Sol), and area postrema (AP). K–N, Sulfolipid storage in forebrain. Vibratome slices are as above. Genotypes are as indicated. Scale bar, 500 μm. In K, the cortex does not display AB-positive structures at this magnification; AB-positive material is present only in the hippocampal fimbria (fi) (mainly within macrophages). In L, the isocortical lamina 5 and the hippocampal CA1 region show prominent staining; moderate staining is seen in the hippocampal CA3 region. In M, AB-positive staining is seen in laminas 5 and 2/3 of the isocortex and in the CA3 region. In the double-trangenic ASA(−/−)mouse (N), increased amounts of AB-positive material are observed in the laminas 5, 6, and 2/3 of the isocortex as well as in the hippocampus including the dentate gyrus (DG). In all CGT- and CST-tg ASA(−/−) mice, increased storage is seen in the thalamus (T).