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Items: 1 to 20 of 76

1.

Bile acid synthetic defects and liver disease.

Bove KE, Daugherty CC, Tyson W, Mierau G, Heubi JE, Balistreri WF, Setchell KD.

Pediatr Dev Pathol. 2000 Jan-Feb;3(1):1-16. Review. No abstract available.

PMID:
10594127
2.

Liver disease caused by disorders of bile acid synthesis.

Bove KE.

Clin Liver Dis. 2000 Nov;4(4):831-48. Review.

PMID:
11232360
3.

Inborn errors of metabolism with consequences for bile acid biosynthesis. A minireview.

Björkhem I.

Scand J Gastroenterol Suppl. 1994;204:68-72. Review.

PMID:
7824882
4.

[3 beta-hydroxy-delta 5-C27-steroid-oxidoreductase/isomerase deficiency].

Kamimura R, Maeda Y, Setoguchi T.

Ryoikibetsu Shokogun Shirizu. 1998;(19 Pt 2):279-81. Review. Japanese. No abstract available.

PMID:
9645061
5.

Neonatal cholestatic liver disease in an Asian patient with a homozygous mutation in the oxysterol 7alpha-hydroxylase gene.

Ueki I, Kimura A, Nishiyori A, Chen HL, Takei H, Nittono H, Kurosawa T.

J Pediatr Gastroenterol Nutr. 2008 Apr;46(4):465-9. doi: 10.1097/MPG.0b013e31815a9911. No abstract available.

PMID:
18367963
6.

[27-Hydroxylase deficiency (cerebrotendinous xanthomatosis)].

Miyamoto K, Setoguchi T.

Ryoikibetsu Shokogun Shirizu. 1998;(19 Pt 2):285-8. Review. Japanese. No abstract available.

PMID:
9645063
7.

Two 7 alpha-hydroxylase enzymes in bile acid biosynthesis.

Schwarz M, Lund EG, Russell DW.

Curr Opin Lipidol. 1998 Apr;9(2):113-8. Review.

PMID:
9559267
8.

Regulation of bile acid synthesis.

Chiang JY.

Front Biosci. 1998 Feb 15;3:d176-93. Review.

PMID:
9450986
9.

[Congenital deficiencies in bile acid synthesis: from diagnosis to treatment].

Vanderpas J.

Rev Med Brux. 1993 Apr;14(4):110-5. French.

PMID:
8502858
10.

Bile acid feeding and hepatic sterol metabolism: effect of deoxycholic acid.

Carulli N, Ponz de Leon M, Zironi F, Iori R, Loria P.

Gastroenterology. 1980 Oct;79(4):637-41. No abstract available.

PMID:
7409383
11.

Seeking ligands for lonely orphan receptors.

Gustafsson JA.

Science. 1999 May 21;284(5418):1285-6. No abstract available.

PMID:
10383308
12.

Molecular genetics of 3beta-hydroxy-Delta5-C27-steroid oxidoreductase deficiency in 16 patients with loss of bile acid synthesis and liver disease.

Cheng JB, Jacquemin E, Gerhardt M, Nazer H, Cresteil D, Heubi JE, Setchell KD, Russell DW.

J Clin Endocrinol Metab. 2003 Apr;88(4):1833-41.

PMID:
12679481
13.

Bile acid synthetic defects and liver disease: a comprehensive review.

Bove KE, Heubi JE, Balistreri WF, Setchell KD.

Pediatr Dev Pathol. 2004 Jul-Aug;7(4):315-34. Epub 2004 Jul 15. Review.

PMID:
15383928
14.
15.

Behold the CHILD, it's only one: CHILD syndrome is not caused by deficiency of 3 beta-hydroxysteroid-Delta 8, Delta 7-isomerase.

Happle R, König A, Grzeschik KH.

Am J Med Genet. 2000 Oct 2;94(4):341-3. No abstract available.

PMID:
11038450
17.

Analysis of HSD3B7 knockout mice reveals that a 3alpha-hydroxyl stereochemistry is required for bile acid function.

Shea HC, Head DD, Setchell KD, Russell DW.

Proc Natl Acad Sci U S A. 2007 Jul 10;104(28):11526-33. Epub 2007 Jun 29.

18.

Bile acid synthesis in the Smith-Lemli-Opitz syndrome: effects of dehydrocholesterols on cholesterol 7alpha-hydroxylase and 27-hydroxylase activities in rat liver.

Honda A, Salen G, Shefer S, Batta AK, Honda M, Xu G, Tint GS, Matsuzaki Y, Shoda J, Tanaka N.

J Lipid Res. 1999 Aug;40(8):1520-8.

19.

Regulation of bile acid biosynthesis.

Vlahcevic ZR, Pandak WM, Stravitz RT.

Gastroenterol Clin North Am. 1999 Mar;28(1):1-25, v. Review.

PMID:
10198776
20.

Genes involved in initial steps of bile acid synthesis.

Björkhem I, Eggertsen G.

Curr Opin Lipidol. 2001 Apr;12(2):97-103. Review.

PMID:
11264980

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