Enhanced Susceptibility of LDL to Oxidative Modification in a CTX Patient:<BR>&mdash; Role of Chenodeoxycholic Acid in Xanthoma Formation &mdash;

DOI Web Site PubMed 被引用文献3件 参考文献38件
  • Kinoshita Makoto
    Department of Internal Medicine, Teikyo University School of Medicine
  • Kawamura Mitsunobu
    Department of Internal Medicine, Tokyo Teishin Hospital
  • Fujita Mineko
    Department of Internal Medicine, Teikyo University School of Medicine
  • Hirota Daisuke
    Department of Internal Medicine, Teikyo University School of Medicine
  • Suda Takaoki
    Department of Internal Medicine, Teikyo University School of Medicine
  • Taki Masanari
    Department of Internal Medicine, Teikyo University School of Medicine
  • Kusano Jyun
    Department of Internal Medicine, Teikyo University School of Medicine
  • Takao Kosuke
    Department of Internal Medicine, Teikyo University School of Medicine
  • Takenaka Hideki
    Department of Internal Medicine, Teikyo University School of Medicine
  • Kubota Shunichiro
    Department of Physiological Chemistry and Nutrition, Faculty of Medicine, University of Tokyo
  • Teramoto Tamio
    Department of Internal Medicine, Teikyo University School of Medicine

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  • Enhanced Susceptibility of LDL to Oxidative Modification in a CTX Patient:<BR>— Role of Chenodeoxycholic Acid in Xanthoma Formation —

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Cerebrotendinous xanthomatosis (CTX) is a rare familial sterol storage disease, causing multiple xanthomas in tendons and the brain. The underlying biochemical defect is a lack of the hepatic mitochondrial cholesterol 27-hydroxylase involved in the normal biosynthesis of bile acid, resulting in reduced biosynthesis of chenodeoxycholic acid (CDCA). It has been reported that administration of CDCA to CTX patients improves neurological disorders and xanthomas of the Achilles tendon. The present study investigated the effect of CDCA on the mechanism of cholesterol accumulation in macrophages, the major cells in xanthoma. The LDL from the patients in this study was significantly more susceptible to oxidative modification than normal LDL, and supplement therapy with CDCA resulted in an improvement in the susceptibility to oxidative modification. In the incubation of CDCA with plasma, 13% of the CDCA added to serum was recovered in the LDL fraction. In addition, supplementation with CDCA enhanced cholesteryl ester transfer protein (CETP) activity and reduced high-density-lipoprotein cholesterol levels in the plasma. This evidence suggests that the multiple xanthomas observed in CTX may be induced by increased oxidized LDL and the low activity of CETP, both of which are caused by a lack of CDCA.

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