OCTN2 / SLC22A5 transports CAR from extracellular space to cytosol

Stable Identifier
Reaction [transition]
Homo sapiens
carnitine [extracellular] + Na+ [extracellular] -> carnitine [cytosol] + Na+ [cytosol]
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OCTN2 (organic cation transporter novel 2, encoded by the SLC22A5 gene) mediates the sodium-dependent transport of CAR (carnitine) from the extracellular space into the 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).

Literature References
PubMed ID Title Journal Year
10559218 Mutations in novel cation transporter (OCTN2), an organic cation/carnitine transporter,with differential effects on the organic cation transport function and the carnitine transport function

Leibach, FH, Seth, P, Ganapathy, V, Huang, W, Wu, X

J Biol Chem 1999
9685390 Molecular and functional identification of sodium ion-dependent, high-affinity human carnitine transporter OCTN2

Oku, A, Shimane, M, Nezu, J, Tamai, I, Yabuuchi, H, Tsuji, A, Sai, Y, Ohashi, R

J Biol Chem 1998
26828774 Carnitine transport and fatty acid oxidation

Pasquali, M, Frigeni, M, Longo, N

Biochim. Biophys. Acta 2016
9706223 Genetic disorders of carnitine metabolism and their nutritional management.

Hoppel, C, Kerner, J

Annu Rev Nutr 1998
Event Information
Catalyst Activity

carnitine transmembrane transporter activity of SLC22A5 [plasma membrane]

Orthologous Events
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