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Peroxisomal fatty acid α- and β -oxidation in humans: enzymology, peroxisomal metabolite transporters and peroxisomal diseases
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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“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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Metadata
- 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)
- Venue
- Biochemical Society Transactions
- Publisher
- Portland Press Ltd.
- 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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