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DMF mediates NRF2-dependent and independent mechanisms on TSS pathway CBS activation DMF activates NRF2, the master regulator for the antioxidative stress response, to regulate the expression of proteins involved in iron hemostasis, and GSH synthesis such as HMOX1, NQO1, FTH, FTL, SLC40A1 in a variety of cellular systems 37

The underlying cause of very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency are mutations in the ACADVL gene [101]

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