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As the rate-limiting enzyme, -BBD shows predominant expression in hepatic, renal, and cerebral tissues ( Cellular carnitine utilization depends on active transmembrane transport mediated by organic cation transporter 2 (OCTN2)

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Coenzyme 10 (Ubidecarenone) is a naturally occurring antioxidant found in body cells, especially in organs with high energy requirements such as the heart and muscles

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This results in the accumulation of electrons which can trigger ROS generation, as well as an accumulation of acyl-CoA which further disrupts mitochondrial function and signaling