Fig. 3
From: Peripheral neuropathy in metachromatic leukodystrophy: current status and future perspective
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