Mitochondrial β-oxidation is the most prominent pathway for fatty acid oxidation but alternative oxidative metabolism exists. Fatty acid ω-oxidation is one of these pathways and forms dicarboxylic acids as products. These dicarboxylic acids are metabolized through peroxisomal β-oxidation representing an alternative pathway, which could potentially limit the toxic effects of fatty acid accumulation. Although dicarboxylic acid metabolism is highly active in liver and kidney, its role in physiology has not been explored in depth. In this review, we summarize the biochemical mechanism of the formation and degradation of dicarboxylic acids through ω- and β-oxidation, respectively. We will discuss the role of dicarboxylic acids in different (patho)physiological states with a particular focus on the role of the intermediates and products generated through peroxisomal β-oxidation. This review is expected to increase the understanding of dicarboxylic acid metabolism and spark future research.
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May 2023
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Cover Image
The cover image is a fluorescent microscopy image showing colocalization of HA-GFP -NPT2A Arg495Cys (green) with FLAG-NHERF1 (red) in OK cells. Treatment with PTH does not change NPT2A Arg495Cys-NHERF1 colocalization at the apical membrane. For more information see the article by Sneddon and colleagues (pp. 685–699) in this issue. The image was provided by Tatyana Mamonova.
Review Article|
May 04 2023
The biochemistry and physiology of long-chain dicarboxylic acid metabolism
Pablo Ranea-Robles
;
1Novo Nordisk Foundation Center for Basic Metabolic Research, University of Copenhagen, Copenhagen, Denmark
Correspondence: Pablo Ranea Robles (pranea88@gmail.com) or Sander Houten (sander.houten@mssm.edu)
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Sander M. Houten
2Department of Genetics and Genomic Sciences, Icahn School of Medicine at Mount Sinai, New York, NY, U.S.A.
Correspondence: Pablo Ranea Robles (pranea88@gmail.com) or Sander Houten (sander.houten@mssm.edu)
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Biochem J (2023) 480 (9): 607–627.
Article history
Received:
February 15 2023
Revision Received:
April 20 2023
Accepted:
April 21 2023
Citation
Pablo Ranea-Robles, Sander M. Houten; The biochemistry and physiology of long-chain dicarboxylic acid metabolism. Biochem J 17 May 2023; 480 (9): 607–627. doi: https://doi.org/10.1042/BCJ20230041
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