A novel pathway for biosynthesis of cholestanol with 7 alpha-hydroxylated C27-steroids as intermediates, and its importance for the accumulation of cholestanol in cerebrotendinous xanthomatosis.

Lignende værker

• Demonstration of 26-hydroxylation of C27-steroids in human skin fibroblasts, and a deficiency of this activity in cerebrotendinous xanthomatosis.
  af: Skrede, S, et al.
  Udgivet: (1986)
• Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study.
  af: Björkhem, I, et al.
  Udgivet: (1983)
• Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid.
  af: Oftebro, H, et al.
  Udgivet: (1980)
• The Metabolism of Cholestanol, Cholesterol, and Bile Acids in Cerebrotendinous Xanthomatosis
  af: Salen, Gerald, et al.
  Udgivet: (1973)
• Biosynthesis of bile acids in man. Hydroxylation of the C27-steroid side chain.
  af: Björkhem, I, et al.
  Udgivet: (1975)