In sequences containing Asp-Pro, the cyclic imide formation can actually cleave the peptide backbone entirely, splitting the peptide into two fragments
This antioxidant effect of L-carnitine was previously reported in patients who underwent rheumatic valve replacement surgery [28] and may be explained by the ability of L-carnitine to activate nuclear factor erythroid 2-related factor 2 (Nrf2)
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Organic acidaemias, including methylmalonic acidaemia, propionic acidaemia, isovaleric acidaemia and multiple carboxylase deficiency occur in ~1 in 21,000 births and typically lead to mild to moderate hyperammonaemia due to competitive inhibition of NAGS 5,6,7,8,9