Chemical structures of lipoic acid and lipoyllysine residue The figure illustrates the chemical structures of lipoic acid in its oxidized and reduced forms, its two enantiomers ( R - and S -lipoic acid), and its covalent attachment to a lysine residue of the E2 component of the -ketoacid dehydrogenase complex (and the H protein of the glycine cleavage system), forming a lipoyllysine moiety
As a result, while not routinely used in clinical practice, LC-MS serves as the reference method against which other testing methods are compared to assess their accuracy
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it occurs in approximately 1 out every 700800 live births [60]