296 related articles for article (PubMed ID: 7596681)
1. 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; 37(4 Pt 1):424-31. PubMed ID: 7596681
[TBL] [Abstract][Full Text] [Related]
2. Biochemical studies of inherited diseases related to abnormal cholesterol metabolism. II: Absence of unusual C28 and C29 bile acid homologs in bile and urine of sitosterolemia.
Ohshima A; Une M; Hoshita T
Hiroshima J Med Sci; 1994 Sep; 43(3):81-6. PubMed ID: 7896564
[TBL] [Abstract][Full Text] [Related]
3. Familial giant cell hepatitis associated with synthesis of 3 beta, 7 alpha-dihydroxy-and 3 beta,7 alpha, 12 alpha-trihydroxy-5-cholenoic acids.
Clayton PT; Leonard JV; Lawson AM; Setchell KD; Andersson S; Egestad B; Sjövall J
J Clin Invest; 1987 Apr; 79(4):1031-8. PubMed ID: 3470305
[TBL] [Abstract][Full Text] [Related]
4. Cholic acid biosynthesis: the enzymatic defect in cerebrotendinous xanthomatosis.
Salen G; Shefer S; Cheng FW; Dayal B; Batta AK; Tint GS
J Clin Invest; 1979 Jan; 63(1):38-44. PubMed ID: 762246
[TBL] [Abstract][Full Text] [Related]
5. 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; 26(6):690-8. PubMed ID: 4031647
[TBL] [Abstract][Full Text] [Related]
6. Variable clinical spectrum of the most common inborn error of bile acid metabolism--3beta-hydroxy-Delta 5-C27-steroid dehydrogenase deficiency.
Subramaniam P; Clayton PT; Portmann BC; Mieli-Vergani G; Hadzić N
J Pediatr Gastroenterol Nutr; 2010 Jan; 50(1):61-6. PubMed ID: 19915491
[TBL] [Abstract][Full Text] [Related]
7. 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; 124(1):217-32. PubMed ID: 12512044
[TBL] [Abstract][Full Text] [Related]
8. Urinary bile alcohol profile in infants with intrahepatic cholestasis: identification of 5beta-cholestane-3alpha,7alpha,24,25-tetrol.
Nakagawa M; Une M; Takenaka S; Kuramoto T; Abukawa D; Iinuma K
Acta Paediatr; 1999 Oct; 88(10):1078-82. PubMed ID: 10565453
[TBL] [Abstract][Full Text] [Related]
9. Inborn errors of bile acid metabolism.
Clayton PT
J Inherit Metab Dis; 1991; 14(4):478-96. PubMed ID: 1749214
[TBL] [Abstract][Full Text] [Related]
10. Delta 4-3-oxosteroid 5 beta-reductase deficiency described in identical twins with neonatal hepatitis. A new inborn error in bile acid synthesis.
Setchell KD; Suchy FJ; Welsh MB; Zimmer-Nechemias L; Heubi J; Balistreri WF
J Clin Invest; 1988 Dec; 82(6):2148-57. PubMed ID: 3198770
[TBL] [Abstract][Full Text] [Related]
11. 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
[TBL] [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; 76(2):744-51. PubMed ID: 4031069
[TBL] [Abstract][Full Text] [Related]
13. Identification of bile alcohols in human bile.
Kuroki S; Shimazu K; Kuwabara M; Une M; Kihira K; Kuramoto T; Hoshita T
J Lipid Res; 1985 Feb; 26(2):230-40. PubMed ID: 3989382
[TBL] [Abstract][Full Text] [Related]
14. Increased plasma bile alcohol glucuronides in patients with cerebrotendinous xanthomatosis: effect of chenodeoxycholic acid.
Batta AK; Salen G; Shefer S; Tint GS; Batta M
J Lipid Res; 1987 Aug; 28(8):1006-12. PubMed ID: 3668385
[TBL] [Abstract][Full Text] [Related]
15. Bile acid therapies applied to patients suffering from cerebrotendinous xanthomatosis.
Koopman BJ; Wolthers BG; van der Molen JC; Waterreus RJ
Clin Chim Acta; 1985 Oct; 152(1-2):115-22. PubMed ID: 4053393
[TBL] [Abstract][Full Text] [Related]
16. Progressive familial intrahepatic cholestasis and inborn errors of bile acid synthesis.
Jankowska I; Socha P
Clin Res Hepatol Gastroenterol; 2012 Jun; 36(3):271-4. PubMed ID: 22609295
[TBL] [Abstract][Full Text] [Related]
17. Chemical synthesis of 5beta-cholestane-3alpha, 7alpha, 24, 25-tetrol and its metabolism in the perfused rabbit liver.
Kuramoto T; Cohen BI; Rothschild MA; Donor DA; Mosbach EH
J Biol Chem; 1978 Jul; 253(13):4688-92. PubMed ID: 659442
[TBL] [Abstract][Full Text] [Related]
18. Synthesis and structure of 26 (or 27)-nor-5 beta-cholestane-3 alpha,7 alpha,12 alpha,24S,25 xi-pentol isolated from the urine and feces of a patient with sitosterolemia and xanthomatosis.
Dayal B; Tint GS; Toome V; Batta AK; Shefer S; Salen G
J Lipid Res; 1985 Mar; 26(3):298-305. PubMed ID: 2985722
[TBL] [Abstract][Full Text] [Related]
19. Delta 4-3-oxosteroid 5 beta-reductase deficiency: failure of ursodeoxycholic acid treatment and response to chenodeoxycholic acid plus cholic acid.
Clayton PT; Mills KA; Johnson AW; Barabino A; Marazzi MG
Gut; 1996 Apr; 38(4):623-8. PubMed ID: 8707100
[TBL] [Abstract][Full Text] [Related]
20. Bile alcohol metabolism in man. Conversion of 5beta-cholestane-3alpha, 7alpha,12alpha, 25-tetrol to cholic acid.
Salen G; Shefer S; Setoguchi T; Mosbach EH
J Clin Invest; 1975 Jul; 56(1):226-31. PubMed ID: 1141434
[TBL] [Abstract][Full Text] [Related]
[Next] [New Search]
