Reaction: OCTN2 / SLC22A5 transports CAR from extracellular space to cytosol
While humans are capable of synthesizing carnitine de novo, the enzyme that catalyzes the last reaction of the biosynthetic pathway is found only in liver and kidney cells, and at very low levels in brain cells. Other tissues that require carnitine, such as muscle, are dependent on transport systems that mediate its export from the liver and uptake by other tissues (Kerner & Hoppel 1998). The specific transport systems responsible for liver export have been characterized biochemically in model organisms but specific transport proteins have not yet been identified. OCTN2 is the major transporter responsible for carnitine uptake in extrahepatic tissues, as demonstrated both by the biochemical characterization of overexpressed recombinant human protein (Tamai et al. 1998) and by the appearance of symptoms of carnitine deficiency in humans lacking a functional SLC22A5 gene (Seth et al. 1999; reviewed by Longo et al. 2016).
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