Cited by 226Metabolism and Genetic DisordersLipid metabolism and biosynthesisPeroxisome Proliferator-Activated Receptors

Peroxisomal fatty acid α- and β -oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases

Wanders, R. J. A., Vreken, P., Ferdinandusse, S., Jansen, G. A., Waterham, H. R., van Roermund, C. W. T., Van Grunsven, E. G.·2001·Biochemical Society Transactions

Abstract

Peroxisomes are subcellular organelles with an indispensable role in cellular metabolism. The importance of peroxisomes for humans is stressed by the existence of a group of genetic diseases in humans in which there is an impairment in one or more peroxisomal functions. Most of these functions have to do with lipid metabolism including the α and β-oxidation of fatty acids. Here we describe the current state of knowledge about peroxisomal fatty acid α- and β-oxidation with particular emphasis on the following: (1) the substrates β-oxidized in peroxisomes; (2) the enzymology of the α- and β-oxidation systems; (3) the permeability properties of the peroxisomal membrane and the role of the different transporters therein; (4) the interaction with other subcellular compartments, including the mitochondria, which are the ultimate site of NADH reoxidation and full degradation of acetyl-CoA to CO2 and water; and (5) the different disorders of peroxisomal α- and β-oxidation.

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Crossref · 10.1042/bst0290250

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  • By binding to ATP, ALDP transports VLCFAcyl-CoA into the peroxisomal matrix, where they are β-oxidized into medium-chain fatty acids (MCFAs) and MCFAcyl-carnitines, propionyl-CoA, and acetyl-CoA.

    “By binding to ATP, ALDP transports VLCFAcyl-CoA into the peroxisomal matrix [143,159,160], where they are β-oxidized into medium-chain fatty acids (MCFAs) and MCFAcyl-carnitines [161], propionyl-CoA, and acetyl-CoA.”

    From Revisiting the Pathogenesis of X-Linked Adrenoleukodystrophy (2025) · cites this paper as [161]

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Authors
Wanders, R. J. A., Vreken, P., Ferdinandusse, S., Jansen, G. A., Waterham, H. R., van Roermund, C. W. T., & Van Grunsven, E. G.
Affiliations
  • Amsterdam UMC Location University of Amsterdam (NL)
  • University of Amsterdam, Academic Medical Centre, Department of Clinical Chemistry and Paediatrics, Emma Children's Hospital, Laboratory for Genetic Metabolic Diseases, P.O. Box 22700, 1100 DE Amsterdam, The Netherlands
Venue
Biochemical Society Transactions
Published
2001-05-01
Volume
29
Issue
2
Pages
250-267
ISSN
  • 0300-5127 (ISSN-L)
  • 1470-8752
OpenAlex
W4231809167
Cited by
225
References
0
Citations by year
2026: 6 · 2025: 7 · 2024: 6 · 2023: 7 · 2022: 6 · 2021: 7 · 2020: 5 · 2019: 5 · 2018: 8 · 2017: 7 · 2016: 4 · 2015: 10 · 2014: 6 · 2013: 7 · 2012: 8
FWCI
10.52
Type
journal article
Language
en
Open access
closed
Locations
Biochemical Society Transactions (publishedVersion)
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
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Peroxisome Proliferator-Activated Receptors
Keywords
peroxisomes, fatty acid beta-oxidation, peroxisomal enzymes, peroxisomal disorders, lipid metabolism, NADH reoxidation, acetyl-CoA oxidation

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