Cited by 192Metabolism and Genetic DisordersLipid metabolism and biosynthesisFatty Acid Research and Health

Very long chain fatty acids in higher animals: a review

Poulos, Alf·1995·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 as retina and spermatozoa. Biosynthesis of VLCFA occurs by carbon chain elongation of shorter chain fatty acid precursors while beta-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 beta-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 beta-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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Crossref · 10.1007/bf02537036

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Authors
Poulos, A.
Affiliations
Women's and Children's Hospital (AU)
Corresponding author
Poulos, Alf
Venue
Lipids
Publisher
Wiley
Published
1995-01
Volume
30
Issue
1
Pages
1-14
ISSN
  • 0024-4201 (ISSN-L)
  • 1558-9307
OpenAlex
W2059163200
Cited by
192
References
240
Citations by year
2026: 2 · 2025: 4 · 2024: 3 · 2023: 5 · 2022: 10 · 2021: 8 · 2020: 8 · 2019: 4 · 2018: 6 · 2017: 11 · 2016: 6 · 2015: 6 · 2014: 5 · 2013: 5 · 2012: 4
FWCI
2.06
Type
journal article
Language
en
Open access
closed
Locations
  • Lipids (publishedVersion)
  • PubMed (publishedVersion)
Funders
  • Hospital Research Foundation
  • National Health and Medical Research Council
  • National Medical Research Council
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
  • Health Sciences › Nursing › Nutrition and Dietetics › Fatty Acid Research and Health
Keywords
very long chain fatty acids, fatty acid synthesis, carbon chain elongation, fatty acid beta-oxidation, peroxisomes, peroxisomal disorders, polyunsaturated fatty acids, brain fatty acids, retina, spermatozoa, fatty acid degradation, human disease
MeSH terms
Animals, Disease, Fatty Acids, Humans, Mammals, Oxidation-Reduction

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