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. 2019 Nov 4;14:240. doi: 10.1186/s13023-019-1220-4

Fig. 3.

Fig. 3

Hypothetical disrupted blood-nerve barrier model leading to leakage of noxious substances from the blood into the endoneurium in metachromatic leukodystrophy (MLD). The release of inflammatory cytokines in MLD, such MCP-1, IL-8 and VEGF, downregulates tight junction proteins, including occludin, claudin-1 and claudin-5 [118]. Consequently, the integrity tight junctions is disrupted, leading to damage of the blood-nerve and leakage of noxious substances from the blood into the endoneurium