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It may be pertinent for clinicians to check carnitine levels with other routine diet therapy lab monitoring (at 3 months, 6 months, then every 6 months) since patients are often asymptomatic, and carnitine deficiency may go undetected if it is not checked

Moreover, as an adapter protein involved in selective autophagy and a target of NRF2, p62/SQSTM1 competitively binds to Keap1, subsequently promoting NRF2 activation

There are different treatments regimes that are either under trial or approved by US-FDA which tabulated in Table 1

Furthermore, these toxic lipid products can react with nucleophilic amino acid residues within various proteins, forming covalent adducts and inducing protein lipoxidation [107]