Oral lecithin and linoleic acid in Friedreich's ataxia: III. Biochemical results.
Lecithin and safflower oil brought about the same changes in serum LAD activity and kinetics in patients with Friedreich's Ataxia as in controls when results of this double-blind crossover study were analyzed according to group assignation. According to functional stages, pretrial LAD activity decreased with advancing severity while Km for lipoamide increased. Lecithin and safflower oil supplements corrected the elevated Km for lipoamide but produced a further reduction in LAD activity. These changes may have been due to the increased intake of linoleic acid, a precursor of lipoic acid, which is present in high percentage in both lecithin and safflower oil. Results of the biochemical study thus agreed with the clinical data gathered during the course of the one-year trial in suggesting that linoleic acid may well have been the active factor through which biochemical and clinical improvement was previously observed in patients with Friedreich's Ataxia supplemented with lecithin.
D000284:Administration, Oral; D008058:Dihydrolipoamide Dehydrogenase / Q000097:blood* / Q000378:metabolism; D005621:Friedreich Ataxia / Q000188:drug therapy* / Q000201:enzymology; D006801:Humans; D007700:Kinetics; D019787:Linoleic Acid; D008041:Linoleic Acids / Q000627:therapeutic use*; D009243:NAD / Q000097:blood; D010713:Phosphatidylcholines / Q000627:therapeutic use*
D008041:Linoleic Acids; D010713:Phosphatidylcholines; D009243:NAD; D019787:Linoleic Acid; D008058:Dihydrolipoamide Dehydrogenase