Carnitine chemical structure

FIGURE 13.2 Chemical structures of L-carnitine (levo-carnitine) and several fatty ACs of different chain length, saturation, or functional groups. 

The primary metabolites of interest in the MS/MS-based NBS panel are a-amino acids, carnitine, and ACs. Figure 13.2 provides generic chemical structures of many ACs while Figure 13.5 provides structures of key amino acids measured in the NBS panel using MS/MS. 

Ascorbic acid s chemical structure makes it an electron donor and therefore a reducing agent. AA has thus been involved in two different biochemical functions redox/ antioxidant properties and enzymatic cofactor. AA has been demonstrated to be an electron donor for different enzymes. Among these enzymes, three are involved in collagen hydroxylation (Bates et al., 1972 Levene et al., 1972). Two are involved in carnitine synthesis (Nelson et al., 1981 Dunn et al., 1984). The remaining are respectively involved in norepinephrine synthesis (Kuo, 1979) and tyrosine synthesis (La Duand Zannoni, 1964). Deficiency in AA has thus been associated with extracellular matrix defects that are probably involved in vascular problems observed in scurvy. 

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