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Ines D, Sonia B, Riadh BM, Amel EG, Slaheddine M, Hamida T, et al

Enzymatic testing Because there is no specific substrate for LCHAD, its activity is determined in the backward reaction by measuring 3-ketopalmitoyl-CoA dehydrogenase activity (LCHAD+SCHAD activity) in the presence and absence of a powerful LCHAD inhibitor (N-ethylmaleimide)