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Carnitine--metabolism and functions.

J Bremer

    Physiological Reviews
    |October 1, 1983
    PubMed
    Summary
    This summary is machine-generated.

    Carnitine is essential for fatty acid metabolism, acting as a carrier for activated fatty acids and acetate across the inner mitochondrial membrane. Its synthesis and regulation are complex, with established roles in various organisms and metabolic pathways.

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    Area of Science:

    • Biochemistry
    • Cellular Metabolism

    Background:

    • Carnitine, initially discovered early in the 20th century, gained recognition for its crucial role in fatty acid metabolism approximately 50 years later.
    • Interest in carnitine's metabolism and functions has significantly grown over the past three decades.
    • While synthesized in most eukaryotes, some insects and newborn animals require carnitine as an essential nutrient (vitamin BT).

    Purpose of the Study:

    • To elucidate the metabolic pathways and essential functions of carnitine.
    • To detail the biosynthesis and physiological roles of carnitine in various organisms.
    • To investigate the transport mechanisms of fatty acids and acetate across mitochondrial membranes mediated by carnitine.

    Main Methods:

    • Described carnitine biosynthesis pathway starting from lysine methylation to butyrobetaine and final hydroxylation in the liver/kidneys.

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  • Identified microorganisms involved in carnitine metabolism and their byproducts.
  • Characterized carnitine's role in transporting activated fatty acids and acetate via acyl-CoA:carnitine acyltransferases across the inner mitochondrial membrane.

    • Main Results:

    • Carnitine is synthesized via lysine methylation, with butyrobetaine as an intermediate, and actively transported to tissues.
    • Microorganisms can metabolize carnitine into various compounds; mammals handle isomers differently, with (-)carnitine excreted unchanged.
    • Carnitine facilitates the transport of activated fatty acids and acetate across the inner mitochondrial membrane through specific acyltransferases and a deduced translocase.

    Conclusions:

    • Carnitine plays a vital, established role in transporting activated acyl groups across the inner mitochondrial membrane, crucial for energy metabolism.
    • Carnitine's synthesis, transport, and metabolic fate exhibit variations across different organisms and cellular compartments like peroxisomes.
    • Further research is needed to fully understand carnitine synthesis regulation and its complete degradation pathways in mammals.