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Defects of the OCNT2 transporter protein result in urinary carnitine wasting, low serum carnitine levels, and decreased intracellular carnitine accumulation.14-16) As a consequence, impaired oxidation of long chain fatty acids leads to deficient energy generation as well as reduced ketogenesis, particularly during fasting or stress, and accumulation of long-chain fatty acids in the cytoplasm of affected tissues