Resveratrol Assists Fatty Acid Oxidation in Myopathic CPTII and VLCAD
Those who suffer with fatty acid oxidation disorders (FAODs) have few treatment options. Most rely on a high-carbohydrate, low-fat diet to control symptoms of myopathy, the painful and dangerous muscle weakness and breakdown common to these disorders. However, scientists have been studying the effects of resveratrol (RSV), a natural, polyphenolic compound, thought to delay the onset of some cancers, as a possible stimulator to the production of protein by mutated genes involved in fatty acid oxidation.
In 2011, scientists in France published what is thought to be the first study of its kind, with RSV on human fibroblasts from CTPII and VLCAD patients. Two patients had serious variants of the disorders but 11 had myopathic, (missense) variants. Missense variants are usually responsible for the less serious expression of these disorders while large deletions, null mutations, and nonsense mutations tend to lead to the more serious and fatal variants. This study is an example of a target-based therapy.
The study, which was published in Human Molecular Genetics in 2011, reported that in their laboratory studies (not in human patients) RSV restored normal fatty acid oxidation flux. This study states: "The results obtained in this study provide evidence for a marked stimulatory effect of RSV on patient cell FA utilization. Indeed, exposure to RSV restores normal FAO capacities in a panel of cells harboring mild CPT2 or VLCAD deficiency." Other studies reported that RSV reduces fat deposits and improves insulin sensitivity in mice.
Since 2011, there has been some published work on this topic. One, "Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts," concluded that RSV and some similar, related compounds, "can improve mitochondrial FAO capacities in human FAO-deficient cells."
These results seem to indicate that RSV may provide a therapeutic pathway for certain FAODs. Its effects on stimulating FAO as well as its ability to limit inflammation and fat deposits may prove valuable to FAOD patients, especially those with myopatic disorders. As with any supplement, caution should be exercised and one's physician should be consulted. The above photo is for illustration only, and is not an endorsement.
Two references follow:
Exposure to resveratrol triggers pharmacological correction of fatty acid utilization in human fatty acid oxidation-deficient fibroblasts Jean Bastin, et al
Hum Mol Genet (2011) 20 (10): 2048-2057.
Stilbenes and resveratrol metabolites improve mitochondrial fatty acid oxidation defects in human fibroblasts
Virginie Aires1, et al
Orphanet Journal of Rare Diseases20149:79
© Aires et al.; licensee BioMed Central Ltd. 2014