Abstract
Although mitochondrial fatty acid β-oxidation (FAO) is considered to be well understood, further elucidation of the pathway continues through evaluation of patients with FAO defects. The FAO pathway can be examined by measuring the 3-hydroxy-fatty acid (3-OHFA) intermediates. We present a unique finding in the study of this pathway: the addition of medium-chain fatty acids to the culture media of fibroblasts results in generation of 3-OHFAs which are two carbons longer than the precursor substrate. Cultured skin fibroblasts from normal and LCHAD-deficient individuals were grown in media supplemented with various chain-length fatty acids. The cell-free medium was analyzed for 3-OHFAs by stable-isotope dilution gas-chromatography/mass-spectrometry. Our finding suggests that a novel carbon chain-length elongation process precedes the oxidation of medium-chain fatty acids. This previously undescribed metabolic step may have important implications for the metabolism of medium-chain triglycerides, components in the dietary treatment of a number of disorders.
Original language | English (US) |
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Pages (from-to) | 193-197 |
Number of pages | 5 |
Journal | Biochemical and Biophysical Research Communications |
Volume | 346 |
Issue number | 1 |
DOIs | |
State | Published - Jul 21 2006 |
Keywords
- Fatty acid oxidation
- Fatty acid synthesis
- Long-chain l-3-hydroxyacyl CoA dehydrogenase
- Medium-chain triglycerides
ASJC Scopus subject areas
- Biophysics
- Biochemistry
- Molecular Biology
- Cell Biology