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147 related items for PubMed ID: 2000261
1. 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]
2. 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]
3. 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]
4. Role of peroxisomes in the biosynthesis of bile acids. Björkhem I, Kase BF, Pedersen JI. Scand J Clin Lab Invest Suppl; 1985 Feb; 177():23-31. PubMed ID: 3865345 [Abstract] [Full Text] [Related]
8. Bile acid profiles in peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency. Clayton PT, Patel E, Lawson AM, Carruthers RA, Collins J. J Clin Invest; 1990 Apr; 85(4):1267-73. PubMed ID: 2318981 [Abstract] [Full Text] [Related]
9. Competitive inhibition of side chain oxidation of 3 alpha, 7 alpha-dihydroxy-5 beta-cholestan-26-oic acid by 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestan-26-oic acid in the hamster. Cass OW, Williams GC, Hanson RF. J Lipid Res; 1980 Feb; 21(2):186-91. PubMed ID: 7373160 [Abstract] [Full Text] [Related]
10. 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]
11. Identification of (24E)-3 alpha,7 alpha-dihydroxy-5 beta-cholest-24-enoic acid and (24R,25S)-3 alpha,7 alpha,24-trihydroxy-5 beta-cholestanoic acid as intermediates in the conversion of 3 alpha,7 alpha-dihydroxy-5 beta-cholestanoic acid to chenodeoxycholic acid in rat liver homogenates. Une M, Inoue A, Kurosawa T, Tohma M, Hoshita T. J Lipid Res; 1994 Apr 11; 35(4):620-4. PubMed ID: 8006516 [Abstract] [Full Text] [Related]
15. 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 11; 24(5):313-9. PubMed ID: 6652941 [Abstract] [Full Text] [Related]
16. Peroxisomal oxidation of the steroid side chain in bile acid formation. Pedersen JI. Biochimie; 1993 Nov 11; 75(3-4):159-65. PubMed ID: 8507677 [Abstract] [Full Text] [Related]
17. 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 11; 87(2):276-83. PubMed ID: 6735073 [Abstract] [Full Text] [Related]
18. [Cell biology from a medical viewpoint. XI. Peroxisomes: unmasking of a hidden organelle by studies of the Zellweger syndrome]. Wanders RJ. Ned Tijdschr Geneeskd; 1992 Jan 25; 136(4):164-8. PubMed ID: 1736126 [No Abstract] [Full Text] [Related]
19. Liver disease caused by failure to racemize trihydroxycholestanoic acid: gene mutation and effect of bile acid therapy. Setchell KD, Heubi JE, Bove KE, O'Connell NC, Brewsaugh T, Steinberg SJ, Moser A, Squires RH. Gastroenterology; 2003 Jan 25; 124(1):217-32. PubMed ID: 12512044 [Abstract] [Full Text] [Related]