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Very long chain fatty acids in higher animals—A review

  • Review
  • Published:
Lipids

Abstract

Fatty acids with greater than 22 carbon atoms (very long chain fatty acids, VLCFA) are present in small amounts in most animal tissues. Saturated and monoenoic VLCFA are major components of brain, while the polyenoic VLCFA occur in significant amounts in certain specialized animal tissues such at retina and spermatozoa. Biosynthesis of VLCFA occurs by carbon chain elongation of shorter chain fatty acid precursors while β-oxidation takes place, almost exclusively in peroxisomes. Mitochondria are unable to oxidize VLCFA because they lack a specific VLCFA coenzyme A synthetase, the first enzyme in the β-oxidation pathway. VLCFA accumulate in the tissues of patients with inherited abnormalities in peroxisomal assembly, and also in individuals with defects in enzymes catalyzing individual reactions along the β-oxidation pathway. It is believed that the accumulation of VLCFA in patient tissues contributes to the severe pathological changes which are a feature of these conditions. However, little is known of the role of VLCFA in normal cellular processes, and of the molecular basis for their contribution to the disease process. The present review provides an outline of the current knowledge of VLCFA including their biosynthesis, degradation, possible function and involvement in human disease.

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Abbreviations

ABC:

ATP-binding cassette

ALD:

adrenoleukodystrophy

ATP:

adenosine triphosphate

CoA:

coenzyme A

FABP:

fatty acid binding protein

TCA:

tricarboxylic acid

TNFα:

tumor necrosis factor α

VLCFA:

very long chain fatty acids

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  1. Department of Chemical Pathology, Women's and Children's Hospital, 72 King William Road, 5006, North Adelaide, South Australia, Australia

    Alf Poulos

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Poulos, A. Very long chain fatty acids in higher animals—A review. Lipids 30, 1–14 (1995). https://doi.org/10.1007/BF02537036

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