Enzyme defect | Total carnitine | Free carnitine | Free:total carnitine ratio |
Carnitine transporter | Very low | Low | Normal |
CPT I | Normal or high | High | High |
Translocase and CPT II | Low | Very low | Low |
VLCAD, LCAD, MCAD, SCAD, LCHAD, SCHAD ETF and ETF dehydrogenase, 2,4-dienoyl-CoA reductase | Normal or low | Low | Normal or low |
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