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Comparison of the maximum respiration for the acylcarnitine ester relative to the equivalent FFA demonstrated that C6 and C8 are significantly more readily oxidized in the free form in the liver, whereas acylcarnitine esters from 12 to 18 carbons long were significantly more readily oxidized than the corresponding free forms (Fig

Therefore, valproic acid, gabapentin, and aminocaproic acid should be avoided in the treatment of D-2-HGA1, on the contrary, benzodiazepines such as chlordiazepoxide, which acts directly on GABAA receptors, can be considered for use (Table 1)

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