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Journal Abstract Search

177 related items for PubMed ID: 3557306

  • 1. 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; 7(2):266-71. PubMed ID: 3557306
    [Abstract] [Full Text] [Related]

  • 2. 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]

  • 3. Biosynthesis of cholestanol from intestinal 7 alpha-hydroxy-4-cholesten-3-one.
    Skrede S, Björkhem I.
    J Biol Chem; 1982 Jul 25; 257(14):8363-7. PubMed ID: 7045120
    [Abstract] [Full Text] [Related]

  • 4. Hepatic 7 alpha-dehydroxylation of bile acid intermediates, and its significance for the pathogenesis of cerebrotendinous xanthomatosis.
    Skrede S, Buchmann MS, Björkhem I.
    J Lipid Res; 1988 Feb 25; 29(2):157-64. PubMed ID: 3367085
    [Abstract] [Full Text] [Related]

  • 5. Metabolism of the cholestanol precursor cholesta-4,6-dien-3-one in different tissues.
    Buchmann MS, Björkhem I, Skrede S.
    Biochim Biophys Acta; 1987 Nov 21; 922(2):111-7. PubMed ID: 3676336
    [Abstract] [Full Text] [Related]

  • 6. On the mechanism of biosynthesis of cholestanol from 7 alpha-hydroxycholesterol.
    Buchmann MS, Björkhem I, Lund AM, Skrede S.
    Scand J Clin Lab Invest Suppl; 1986 Nov 21; 184():41-6. PubMed ID: 3109015
    [Abstract] [Full Text] [Related]

  • 7. 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 21; 75(2):448-55. PubMed ID: 3919058
    [Abstract] [Full Text] [Related]

  • 8. 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]

  • 9. A novel route for the biosynthesis of cholestanol, and its significance for the pathogenesis of cerebrotendinous xanthomatosis.
    Skrede S, Bjørkhem I.
    Scand J Clin Lab Invest Suppl; 1985 Sep 20; 177():15-21. PubMed ID: 3865344
    [Abstract] [Full Text] [Related]

  • 10. Potential bile acid precursors in plasma--possible indicators of biosynthetic pathways to cholic and chenodeoxycholic acids in man.
    Axelson M, Sjövall J.
    J Steroid Biochem; 1990 Aug 28; 36(6):631-40. PubMed ID: 2214780
    [Abstract] [Full Text] [Related]

  • 11. Cerebrotendinous xanthomatosis: defective liver mitochondrial hydroxylation of chenodeoxycholic acid precursors.
    Oftebro H, Björkhem I, Størmer FC, Pedersen JI.
    J Lipid Res; 1981 May 28; 22(4):632-40. PubMed ID: 6792308
    [Abstract] [Full Text] [Related]

  • 12. 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 28; 76(2):744-51. PubMed ID: 4031069
    [Abstract] [Full Text] [Related]

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  • 14. Cerebrotendinous xanthomatosis: a rare disease with diverse manifestations.
    Moghadasian MH, Salen G, Frohlich JJ, Scudamore CH.
    Arch Neurol; 2002 Apr 28; 59(4):527-9. PubMed ID: 11939886
    [Abstract] [Full Text] [Related]

  • 15. Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid.
    Pedley TA, Emerson RG, Warner CL, Rowland LP, Salen G.
    Ann Neurol; 1985 Oct 28; 18(4):517-8. PubMed ID: 4073846
    [Abstract] [Full Text] [Related]

  • 16. 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 28; 22(2):191-200. PubMed ID: 7017048
    [Abstract] [Full Text] [Related]

  • 17. Treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid in three siblings.
    Chang WN, Kuriyama M, Chee EC.
    J Formos Med Assoc; 1994 Mar 28; 93(3):256-9. PubMed ID: 7920069
    [Abstract] [Full Text] [Related]

  • 18. Increased concentrations of cholestanol and apolipoprotein B in the cerebrospinal fluid of patients with cerebrotendinous xanthomatosis. Effect of chenodeoxycholic acid.
    Salen G, Berginer V, Shore V, Horak I, Horak E, Tint GS, Shefer S.
    N Engl J Med; 1987 May 14; 316(20):1233-8. PubMed ID: 3106810
    [Abstract] [Full Text] [Related]

  • 19. [Laboratory diagnosis of a rare congenital neurodegenerative disease: cerebrotendinous xanthomatosis].
    Varga VE, Katkó M, Harangi J, Balogh I, Kapás I, Madar L, Seres I, Molnár MJ, Paragh G, Kovács GG, Harangi M.
    Orv Hetil; 2014 May 25; 155(21):811-6. PubMed ID: 24836315
    [Abstract] [Full Text] [Related]

  • 20. High levels of plant sterols and cholesterol precursors in cerebrotendinous xanthomatosis.
    Kuriyama M, Fujiyama J, Kasama T, Osame M.
    J Lipid Res; 1991 Feb 25; 32(2):223-9. PubMed ID: 2066659
    [Abstract] [Full Text] [Related]

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