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2. Biosynthesis of bile acids in cerebrotendinous xanthomatosis. Relationship of bile acid pool sizes and synthesis rates to hydroxylations at C-12, C-25, and C-26. Salen G, Shefer S, Tint GS, Nicolau G, Dayal B, Batta AK. J Clin Invest; 1985 Aug; 76(2):744-51. PubMed ID: 4031069 [Abstract] [Full Text] [Related]
3. Assay of intermediates in bile acid biosynthesis using isotope dilution--mass spectrometry: hepatic levels in the normal state and in cerebrotendinous xanthomatosis. Björkhem I, Oftebro H, Skrede S, Pedersen JI. J Lipid Res; 1981 Feb; 22(2):191-200. PubMed ID: 7017048 [Abstract] [Full Text] [Related]
4. Transformation of 4-cholesten-3-one and 7 alpha-hydroxy-4-cholesten-3-one into cholestanol and bile acids in cerebrotendinous xanthomatosis. Salen G, Shefer S, Tint GS. Gastroenterology; 1984 Aug; 87(2):276-83. PubMed ID: 6735073 [Abstract] [Full Text] [Related]
5. Role of the 26-hydroxylase in the biosynthesis of bile acids in the normal state and in cerebrotendinous xanthomatosis. An in vivo study. Björkhem I, Fausa O, Hopen G, Oftebro H, Pedersen JI, Skrede S. J Clin Invest; 1983 Jan; 71(1):142-8. PubMed ID: 6848555 [Abstract] [Full Text] [Related]
6. Cerebrotendinous xanthomatosis: a defect in mitochondrial 26-hydroxylation required for normal biosynthesis of cholic acid. Oftebro H, Björkhem I, Skrede S, Schreiner A, Pederson JI. J Clin Invest; 1980 Jun; 65(6):1418-30. PubMed ID: 7410549 [Abstract] [Full Text] [Related]
7. Accumulation of 7 alpha-hydroxy-4-cholesten-3-one and cholesta-4,6-dien-3-one in patients with cerebrotendinous xanthomatosis: effect of treatment with chenodeoxycholic acid. Björkhem I, Skrede S, Buchmann MS, East C, Grundy S. Hepatology; 1987 Jun; 7(2):266-71. PubMed ID: 3557306 [Abstract] [Full Text] [Related]
8. Biosynthesis of chenodeoxycholic acid: side-chain hydroxylation of 5 beta-cholestane-3 alpha, 7 alpha-diol by subcellular fractions of guinea pig liver. Hoshita N, Shefer S, Cheng FW, Dayal B, Batta AK, Tint GS, Salen G, Mosbach EH. Lipids; 1978 Dec; 13(12):961-5. PubMed ID: 750834 [Abstract] [Full Text] [Related]
9. An in vivo evaluation of the quantitative significance of several potential pathways to cholic and chenodeoxycholic acids from cholesterol in man. Swell L, Gustafsson J, Schwartz CC, Halloran LG, Danielsson H, Vlahcevic ZR. J Lipid Res; 1980 May; 21(4):455-66. PubMed ID: 7381336 [Abstract] [Full Text] [Related]
10. Isolation of 5 alpha-cholestane-3 beta, 7 alpha-diol from bile of patients with cerebrotendinous xanthomatosis. Inefficiency of this steroid as a precursor to cholestanol. Björkhem I, Buchmann MS, Skrede S. Biochim Biophys Acta; 1983 Sep 20; 753(2):220-6. PubMed ID: 6412759 [Abstract] [Full Text] [Related]
12. Biochemical abnormalities in cerebrotendinous xanthomatosis. Salen G, Shefer S, Berginer V. Dev Neurosci; 1991 Sep 20; 13(4-5):363-70. PubMed ID: 1817043 [Abstract] [Full Text] [Related]
13. On the substrate specificity of human CYP27A1: implications for bile acid and cholestanol formation. Norlin M, von Bahr S, Bjorkhem I, Wikvall K. J Lipid Res; 2003 Aug 20; 44(8):1515-22. PubMed ID: 12777473 [Abstract] [Full Text] [Related]
18. 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. Skrede S, Björkhem I, Buchmann MS, Hopen G, Fausa O. J Clin Invest; 1985 Feb 20; 75(2):448-55. PubMed ID: 3919058 [Abstract] [Full Text] [Related]