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Multiple acyl-CoA dehydrogenase deficiency: a rare cause of acidosis with an increased anion gap
A.S. Grice, T.E. Peck British Journal of Anaesthesia Volume 86, Issue 3, Pages (March 2001) DOI: /bja/ Copyright © 2001 British Journal of Anaesthesia Terms and Conditions
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Fig 1 The pathways involved in normal β-oxidation of fatty acids. In order to metabolize fatty acids it is first necessary to transport them into the mitochondria. Once inside the mitochondria then β-oxidation can follow. MC-DCA, medium chain dicarboxylic acid; SC-DCA, short chain dicarboxylic acid; CoA-SH, coenzyme A; ETF, electron transfer flavoprotein; ETF-QO, electron transfer flavoprotein ubiquinone, (coenzyme Q) oxidoreductase. British Journal of Anaesthesia , DOI: ( /bja/ ) Copyright © 2001 British Journal of Anaesthesia Terms and Conditions
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Fig 2 Alternative pathways available for handling fatty acids. When the pathways for β-oxidation are unavailable, cytoplasmic peroxisomes and microsomes are utilized for ω-oxidation of fatty acids. This produces short chain dicarboxylic acids that are eliminated in the urine. British Journal of Anaesthesia , DOI: ( /bja/ ) Copyright © 2001 British Journal of Anaesthesia Terms and Conditions
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