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. Author manuscript; available in PMC: 2014 Mar 6.
Published in final edited form as: Neuron. 2013 Mar 6;77(5):886–898. doi: 10.1016/j.neuron.2013.01.012

Figure 7. Altered SC lipid metabolism accompanied by the toxic accumulation of lipid intermediates induces axonal degeneration and demyelination in mitochondria-related peripheral neuropathies.

Figure 7

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