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The OCTN2 carnitine transporter mediates high-affinity carnitine transport Defective OCTN2 activity reduces carnitine levels and impairs fatty acid oxidation Missense mutations in OCTN2 define the function of selected transporter domains Defects in carnitine biosynthesis do not reduce carnitine levels Acknowledgments This work was supported in part by NIH grant DK 53824

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11-deoxycorticosterone) exhibits some mineralocorticoid action but only about 3% of that of aldosterone

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Neither the enzymatic mechanism of CntA/CntB, nor the reaction sequence of MSA-DH relate to this atom

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