The DOI matches its Crossref record; 9 of the 9 references it cites were found in a registry or an index.
The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders
Open accessAbstract
Mitochondrial fatty acid β-oxidation (FAO) is the major pathway for the degradation of fatty acids and is essential for maintaining energy homeostasis in the human body. Fatty acids are a crucial energy source in the postabsorptive and fasted states when glucose supply is limiting. But even when glucose is abundantly available, FAO is a main energy source for the heart, skeletal muscle, and kidney. A series of enzymes, transporters, and other facilitating proteins are involved in FAO. Recessively inherited defects are known for most of the genes encoding these proteins. The clinical presentation of these disorders may include hypoketotic hypoglycemia, (cardio)myopathy, arrhythmia, and rhabdomyolysis and illustrates the importance of FAO during fasting and in hepatic and (cardio)muscular function. In this review, we present the current state of knowledge on the biochemistry and physiological functions of FAO and discuss the pathophysiological processes associated with FAO disorders.
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Energy metabolism in health and diseases
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A Fatty Acid Oxidation-Dependent Metabolic Shift Regulates Adult Neural Stem Cell Activity
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Disorders of mitochondrial long-chain fatty acid oxidation and the carnitine shuttle
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Metadata
- Authors
- Houten, S. M., Violante, S., Ventura, F. V., & Wanders, R. J. A.
- Venue
- Annual Review of Physiology
- Publisher
- Annual Reviews
- Published
- 2016-02-10
- Volume
- 78
- Issue
- 1
- Pages
- 23-44
- ISSN
- 0066-4278 (ISSN-L)
- 1545-1585
- OpenAlex
- W1963449298
- Cited by
- 920
- References
- 9
- FWCI
- 2.94
- Type
- journal article
- Language
- en
- Open access
- green
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- Houten, S. M., Violante, S., Ventura, F. V., & Wanders, R. J. A. (2016). The Biochemistry and Physiology of Mitochondrial Fatty Acid β-Oxidation and Its Genetic Disorders. Annual Review of Physiology. https://doi.org/10.1146/annurev-physiol-021115-105045
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