A general introduction to the biochemistry of mitochondrial fatty acid β‐oxidation
Open accessAbstract
Over the years, the mitochondrial fatty acid β-oxidation (FAO) pathway has been characterised at the biochemical level as well as the molecular biological level. FAO plays a pivotal role in energy homoeostasis, but it competes with glucose as the primary oxidative substrate. The mechanisms behind this so-called glucose-fatty acid cycle operate at the hormonal, transcriptional and biochemical levels. Inherited defects for most of the FAO enzymes have been identified and characterised and are currently included in neonatal screening programmes. Symptoms range from hypoketotic hypoglycaemia to skeletal and cardiac myopathies. The pathophysiology of these diseases is still not completely understood, hampering optimal treatment. Studies of patients and mouse models will contribute to our understanding of the pathogenesis and will ultimately lead to better treatment.
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CPT1 itself is allosterically inhibited by the fatty acid synthesis intermediate malonyl-CoA.
“CPT1 itself is allosterically inhibited by the fatty acid synthesis intermediate malonyl-CoA (41, 63).”
From Regulation and function of the mammalian tricarboxylic acid cycle (2023) · cites this paper as [63]
CPT1 forms acylcarnitines from fatty acyl CoAs; acylcarnitines are shuttled into mitochondria through SLC25A20; CPT2 liberates fatty acid from carnitine, initiating FAO.
“The outer mitochondrial membrane (OMM) enzyme carnitine palmitoyl transferase 1 (CPT1) forms acylcarnitines from fatty acyl CoAs.50 Acylcarnitines are shuttled into mitochondria through the carnitine–acylcarnitine translocase (SLC25A20) in the IMM. CPT2 liberates FA from carnitine, initiating FAO.51”
From The multifaceted contributions of mitochondria to cellular metabolism (2018) · cites this paper as [51]
Mitochondrial fatty acid oxidation's specific role in human energy homeostasis became obvious from severe, life-threatening hypoketotic hypoglycemia elicited by prolonged fasting in children with diminished function of the pathway.
“Mitochondrial fatty acid oxidation was understood to be an efficient bioenergetic pathway, but its specific role in human energy homeostasis became obvious from the severe, life-threatening hypoketotic hypoglycemia elicited by prolonged fasting in children with diminished function of the pathway (Houten and Wanders, 2010).”
From Cellular Metabolism and Disease: What Do Metabolic Outliers Teach Us? (2012) · cites this paper as Houten and Wanders, 2010
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Hennrich, M. L., Romanov, N., Horn, P., Jaeger, S., Eckstein, V., Steeples, V., Ye, F., Ding, X., Poisa-Beiro, L., Lai, M. C., et al. (2018) · Nature Communications · doi:10.1038/s41467-018-06353-4 · Cited by 101 · see citing works
The role of mitochondria in rheumatic diseases
Becker, Y. L. C., Duvvuri, B., Fortin, P. R., Lood, C., & Boilard, E. (2022) · Nature Reviews Rheumatology · doi:10.1038/s41584-022-00834-z · Cited by 81 · see citing works
- Authors
- Houten, S. M., & Wanders, R. J. A.
- Affiliations
- Amsterdam UMC Location University of Amsterdam (NL)
- Corresponding author
- Houten, Sander Michel
- Venue
- Journal of Inherited Metabolic Disease
- Publisher
- Wiley
- Published
- 2010-03-02
- Volume
- 33
- Issue
- 5
- Pages
- 469-477
- ISSN
- 0141-8955 (ISSN-L)
- 1573-2665
- PMC
- PMC2950079
- OpenAlex
- W2043054174
- Cited by
- 1,020
- References
- 80
- Citations by year
- 2026: 109 · 2025: 108 · 2024: 91 · 2023: 79 · 2022: 78 · 2021: 94 · 2020: 110 · 2019: 64 · 2018: 63 · 2017: 58 · 2016: 33 · 2015: 38 · 2014: 28 · 2013: 27 · 2012: 24
- FWCI
- 2.16
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Inherited Metabolic Disease (publishedVersion, cc-by-nc)
- PubMed Central (publishedVersion, cc-by-nc)
- PubMed (publishedVersion)
- UvA-DARE (University of Amsterdam) (publishedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion, other-oa)
- Data Archiving and Networked Services (DANS) (submittedVersion)
- Pure Amsterdam UMC (submittedVersion)
- Funders
- Nederlandse Organisatie voor Wetenschappelijk Onderzoek
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Biochemistry › Lipid metabolism and biosynthesis
- Keywords
- mitochondrial beta-oxidation, glucose-fatty acid cycle, energy homeostasis, newborn screening, skeletal myopathy, cardiomyopathy, mouse model
- MeSH terms
- Animals, Disease Models, Animal, Energy Metabolism, Fatty Acids, Genotype, Homeostasis, Humans, Lipid Metabolism, Inborn Errors, Mitochondria, Oxidation-Reduction, Phenotype, Mitochondrial Diseases, Mice
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