Figure 7. Altered SC lipid metabolism accompanied by the toxic accumulation of lipid intermediates induces axonal degeneration and demyelination in mitochondria-related peripheral neuropathies.
Diagram describing the proposed model for how activation of a maladaptive integrated stress response as well as a shift in lipid metabolism away from lipid synthesis and towards lipid oxidation secondary to SC mitochondrial dysfunction may contribute to the pathology in peripheral neuropathies. HRI: heme-regulated inhibitor kinase; CB: cerebrosides; ST: sulfatides; AC: acylcarnitine
