Sites and regulation of carnitine biosynthesis in mammals.

  title={Sites and regulation of carnitine biosynthesis in mammals.},
  author={Charles J. Rebouche},
  journal={Federation proceedings},
  volume={41 12},
Although the pathway of carnitine biosynthesis in mammals is known, the location of active synthesis of carnitine and regulation of the pathway have not been clearly defined. Studies in several laboratories have shown that the enzymes that collectively convert epsilon-N-trimethyllysine (epsilon-N-TML) to gamma-butyrobetaine are found in all tissues studied in rats and humans, but distribution of the final enzyme of the pathway, gamma-butyrobetaine, 2-oxoglutarate dioxygenase (gamma… CONTINUE READING


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