Peroxisomes, lipid metabolism and lipotoxicity
Abstract
Peroxisomes play an essential role in cellular lipid metabolism as exemplified by the existence of a number of genetic diseases in humans caused by the impaired function of one of the peroxisomal enzymes involved in lipid metabolism. Key pathways in which peroxisomes are involved include: (1.) fatty acid beta-oxidation; (2.) etherphospholipid biosynthesis, and (3.) fatty acid alpha-oxidation. In this paper we will describe these different pathways in some detail and will provide an overview of peroxisomal disorders of metabolism and in addition discuss the toxicity of the intermediates of peroxisomal metabolism as they accumulate in the different peroxisomal deficiencies.
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CROT is a peroxisomal enzyme that couples short chain fatty acids to carnitine and allows their entry into the mitochondrial matrix.
“CROT, sometimes abbreviated COT, is a peroxisomal enzyme that couples short chain fatty acids to carnitine and thereby allows their entry in the mitochondrial matrix.”
From Expression of miR-33 from an SREBP2 Intron Inhibits Cholesterol Export and Fatty Acid Oxidation* (2010) · cites this paper as [29]
Peroxisomal β-oxidation is limited to degradation of long- and medium-chain fatty acids and cannot oxidize short chain fatty acids.
“Peroxisomal -oxidation is limited to the degradation of long-and medium-chain fatty acids as it is not able to oxidize short chain fatty acids (29).”
From Expression of miR-33 from an SREBP2 Intron Inhibits Cholesterol Export and Fatty Acid Oxidation* (2010) · cites this paper as [29]
CROT is localized to the peroxisomes, it serves to transfer remnants from peroxisomal β-oxidation to the mitochondria and therefore fuels mitochondria with fatty acids independently of CPT1A.
“Although CROT is localized to the peroxisomes, it serves to transfer remnants from peroxisomal -oxidation to the mitochondria and therefore fuels mitochondria with fatty acids independently of CPT1A (29).”
From Expression of miR-33 from an SREBP2 Intron Inhibits Cholesterol Export and Fatty Acid Oxidation* (2010) · cites this paper as [29]
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References 75 · Cited by 169
- Authors
- Wanders, R. J. A., Ferdinandusse, S., Brites, P., & Kemp, S.
- Affiliations
- Amsterdam UMC Location University of Amsterdam (NL)
- Instituto de Biologia Molecular e Celular (PT)
- Corresponding author
- Wanders, R.J.A.
- Venue
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids
- Publisher
- Elsevier BV
- Published
- 2010-03
- Volume
- 1801
- Issue
- 3
- Pages
- 272-280
- ISSN
- 1388-1981 (ISSN-L)
- OpenAlex
- W2068711980
- Cited by
- 169
- References
- 75
- Citations by year
- 2026: 6 · 2025: 5 · 2024: 9 · 2023: 5 · 2022: 7 · 2021: 15 · 2020: 17 · 2019: 5 · 2018: 11 · 2017: 8 · 2016: 5 · 2015: 8 · 2014: 16 · 2013: 12 · 2012: 16
- FWCI
- 1.66
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids (publishedVersion)
- PubMed (publishedVersion)
- Pure Amsterdam UMC (publishedVersion)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Funders
- Association Européenne contre les Leucodystrophies
- 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, lipid metabolism, lipotoxicity, peroxisomal disorders, fatty acid beta-oxidation, peroxisomal enzyme deficiency
- MeSH terms
- Animals, Disease Models, Animal, Fatty Acids, Humans, Lipid Peroxidation, Peroxisomes, Lipid Metabolism Disorders, Metabolic Networks and Pathways
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