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In the last few years mouse models have been developed to study the inborn errors of fatty acid oxidation with phenotypes representative of the human diseases (as shown in Table 1)

Indirect effects LC and ALC have also been reported to affect the HPG axis to promote reproductive hormone secretion [16, 17, 76]

Second, we did not measure the other gene expression related to fat metabolism

3.2 Carnitine deficiency across the course of diet therapy Of the 47 patients with a normal free carnitine level at baseline prior to starting MAD, 18 (38.3%) patients developed free carnitine deficiency during the course of diet therapy