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203 related items for PubMed ID: 3761102

  • 1. Bile acids and bile alcohols in two patients with Zellweger (cerebro-hepato-renal) syndrome.
    Délèze G, Björkhem I, Karlaganis G.
    J Pediatr Gastroenterol Nutr; 1986; 5(5):701-10. PubMed ID: 3761102
    [Abstract] [Full Text] [Related]

  • 2. Defective peroxisomal cleavage of the C27-steroid side chain in the cerebro-hepato-renal syndrome of Zellweger.
    Kase BF, Björkhem I, Hågå P, Pedersen JI.
    J Clin Invest; 1985 Feb; 75(2):427-35. PubMed ID: 3973012
    [Abstract] [Full Text] [Related]

  • 3. In vivo and vitro studies on formation of bile acids in patients with Zellweger syndrome. Evidence that peroxisomes are of importance in the normal biosynthesis of both cholic and chenodeoxycholic acid.
    Kase BF, Pedersen JI, Strandvik B, Björkhem I.
    J Clin Invest; 1985 Dec; 76(6):2393-402. PubMed ID: 4077985
    [Abstract] [Full Text] [Related]

  • 4. Zellweger's cerebro-hepato-renal syndrome--variations in expressivity and in defects of bile acid synthesis.
    Gustafsson J, Gustavson KH, Karlaganis G, Sjövall J.
    Clin Genet; 1983 Nov; 24(5):313-9. PubMed ID: 6652941
    [Abstract] [Full Text] [Related]

  • 5. Role of peroxisomes in the biosynthesis of bile acids.
    Björkhem I, Kase BF, Pedersen JI.
    Scand J Clin Lab Invest Suppl; 1985 Nov; 177():23-31. PubMed ID: 3865345
    [Abstract] [Full Text] [Related]

  • 6. Importance of peroxisomes in the formation of chenodeoxycholic acid in human liver. Metabolism of 3 alpha,7 alpha-dihydroxy-5 beta-cholestanoic acid in Zellweger syndrome.
    Kase BF, Pedersen JI, Wathne KO, Gustafsson J, Björkhem I.
    Pediatr Res; 1991 Jan; 29(1):64-9. PubMed ID: 2000261
    [Abstract] [Full Text] [Related]

  • 7. Deficient cholesterol side chain oxidation in patients without peroxisomes (Zellweger syndrome): evidence for the involvement of peroxisomes in bile acid synthesis in man.
    Wanders RJ, Schutgens RB, Heymans HS.
    Clin Chim Acta; 1987 Feb 15; 162(3):295-301. PubMed ID: 3568406
    [Abstract] [Full Text] [Related]

  • 8. Biosynthesis of bile acids in man. An in vivo evaluation of the conversion of R and S 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic and 3 alpha, 7 alpha, 12 alpha-24 xi-tetrahydroxy-5 beta-cholestanoic acids to cholic acid.
    Swell L, Gustafsson J, Danielsson H, Schwartz CC, Vlahcevic ZR.
    J Biol Chem; 1981 Jan 25; 256(2):912-6. PubMed ID: 7005227
    [Abstract] [Full Text] [Related]

  • 9. In vitro formation of bile acids from di- and trihydroxy-5 beta-cholestanoic acid in human liver peroxisomes.
    Kase BF, Prydz K, Björkhem I, Pedersen JI.
    Biochim Biophys Acta; 1986 Jun 11; 877(1):37-42. PubMed ID: 3013317
    [Abstract] [Full Text] [Related]

  • 10. Identification of 3 alpha, 6 alpha, 7 alpha, 12 alpha-tetrahydroxy-5 beta-cholestanoic acid in Zellweger's syndrome.
    Une M, Kisaka N, Yoshii M, Hoshita T.
    J Biochem; 1989 Sep 11; 106(3):501-4. PubMed ID: 2606901
    [Abstract] [Full Text] [Related]

  • 11. Biliary bile acids, bile alcohols, and sterols of Alligator mississippiensis.
    Tint GS, Dayal B, Batta AK, Shefer S, Joanen T, McNease L, Salen G.
    J Lipid Res; 1980 Jan 11; 21(1):110-7. PubMed ID: 7354247
    [Abstract] [Full Text] [Related]

  • 12. Biosynthesis of cholic acid in rat liver: formation of cholic acid from 3 alpha, 7 alpha, 12 alpha-trihydroxy- and 3 alpha, 7 alpha, 12 alpha, 24-tetrahydroxy-5 beta-cholestanoic acids.
    Gustafsson J.
    Lipids; 1980 Feb 11; 15(2):113-21. PubMed ID: 7374359
    [Abstract] [Full Text] [Related]

  • 13. Identification of new C27 and C24 bile acids in the bile of Alligator mississippiensis.
    Kihira K, Okamoto A, Hoshita T.
    J Biochem; 1987 Jun 11; 101(6):1377-84. PubMed ID: 3667554
    [Abstract] [Full Text] [Related]

  • 14. Human hepatoblastoma cells (HepG2) and rat hepatoma cells are defective in important enzyme activities in the oxidation of the C27 steroid side chain in bile acid formation.
    Farrants AK, Nilsson A, Pedersen JI.
    J Lipid Res; 1993 Dec 11; 34(12):2041-50. PubMed ID: 8301225
    [Abstract] [Full Text] [Related]

  • 15. Occurrence of both (25R)- and (25S)-3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acids in urine from an infant with Zellweger's syndrome.
    Une M, Tazawa Y, Tada K, Hoshita T.
    J Biochem; 1987 Dec 11; 102(6):1525-30. PubMed ID: 3448094
    [Abstract] [Full Text] [Related]

  • 16. Effect of chenodeoxycholic acid on biliary and urinary bile acids and bile alcohols in cerebrotendinous xanthomatosis; monitoring by high performance liquid chromatography.
    Batta AK, Shefer S, Batta M, Salen G.
    J Lipid Res; 1985 Jun 11; 26(6):690-8. PubMed ID: 4031647
    [Abstract] [Full Text] [Related]

  • 17. Bile acid abnormalities and the diagnosis of cerebro-hepato-renal syndrome (Zellweger syndrome).
    Eyssen H, Eggermont E, van Eldere J, Jaeken J, Parmentier G, Janssen G.
    Acta Paediatr Scand; 1985 Jul 11; 74(4):539-44. PubMed ID: 4024924
    [Abstract] [Full Text] [Related]

  • 18. Familial giant cell hepatitis with low bile acid concentrations and increased urinary excretion of specific bile alcohols: a new inborn error of bile acid synthesis?
    Clayton PT, Casteels M, Mieli-Vergani G, Lawson AM.
    Pediatr Res; 1995 Apr 11; 37(4 Pt 1):424-31. PubMed ID: 7596681
    [Abstract] [Full Text] [Related]

  • 19. Inborn errors of bile acid metabolism.
    Clayton PT.
    J Inherit Metab Dis; 1991 Apr 11; 14(4):478-96. PubMed ID: 1749214
    [Abstract] [Full Text] [Related]

  • 20. Rapid diagnosis of Zellweger syndrome and infantile Refsum's disease by fast atom bombardment--mass spectrometry of urine bile salts.
    Lawson AM, Madigan MJ, Shortland D, Clayton PT.
    Clin Chim Acta; 1986 Dec 15; 161(2):221-31. PubMed ID: 2433077
    [Abstract] [Full Text] [Related]

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