| dc.description.abstract | Phytanic acid is one of many branched-chained fatty acids that humans and animals consume on a daily basis, but impaired metabolism of this compound and other branched-chain lipids such as cholesterol leads to toxicity. This toxicity may cause a variety of metabolic disruptions, but the full mechanism behind the branched-chain lipid accumulation has not been discovered. Additionally, phytanic acid binds to receptors similarly to fibrates, less toxic drugs developed from branched chain fatty acids like phytanic acid, which are used for controlling high triglycerides and cholesterol. Thus, errors in phytanic acid processing can cause similar problems in fibrate processing. In vitro studies showed that Sterol Carrier Protein-2 (SCP-2) and Sterol Carrier Protein-x (SCP-x), encoded by the same Scp-2 gene through different initiation sites, facilitate branched-chain lipid metabolism. Since their physiological impact is not yet clear, SCP-2/SCP-x gene ablated (“double knockout”, DKO) and wild-type mice were fed control versus high phytol diet. Lipid analysis, real-time PCRs and western blotting of livers and serum demonstrated that SCP-2/SCP-x markedly impact branched-chain lipid metabolism—leading to cholesterol and lipid accumulation. | en |
