(Q38333581)

2 September 2017

title

Disrupted coordinate regulation of farnesoid X receptor target genes in a patient with cerebrotendinous xanthomatosis (English)

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2 September 2017

Akira Honda

1

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2 September 2017

Gerald Salen

2

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2 September 2017

Yasushi Matsuzaki

3

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2 September 2017

Ashok K Batta

4

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2 September 2017

Guorong Xu

5

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2 September 2017

Takeshi Hirayama

6

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2 September 2017

G Stephen Tint

7

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2 September 2017

Mikio Doy

8

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2 September 2017

Sarah Shefer

9

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2 September 2017

language of work or name

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1 December 2004

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Journal of Lipid Research

2 September 2017

published in

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2 September 2017

volume

46

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2 September 2017

issue

2

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2 September 2017

page(s)

287-296

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Bile acid metabolism and its role in human cholesterol balance

2 September 2017

cites work

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A regulatory cascade of the nuclear receptors FXR, SHP-1, and LRH-1 represses bile acid biosynthesis

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Targeted disruption of the nuclear receptor FXR/BAR impairs bile acid and lipid homeostasis.

21 January 2018

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Human bile salt export pump promoter is transactivated by the farnesoid X receptor/bile acid receptor.

21 January 2018

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Frameshift and splice-junction mutations in the sterol 27-hydroxylase gene cause cerebrotendinous xanthomatosis in Jews or Moroccan origin

21 January 2018

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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

21 January 2018

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Side chain hydroxylations in bile acid biosynthesis catalyzed by CYP3A are markedly up-regulated in Cyp27-/- mice but not in cerebrotendinous xanthomatosis.

21 January 2018

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Long-term treatment of cerebrotendinous xanthomatosis with chenodeoxycholic acid

21 January 2018

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Disruption of the sterol 27-hydroxylase gene in mice results in hepatomegaly and hypertriglyceridemia. Reversal by cholic acid feeding.

21 January 2018

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Markedly reduced bile acid synthesis but maintained levels of cholesterol and vitamin D metabolites in mice with disrupted sterol 27-hydroxylase gene

21 January 2018

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Hydrolysis of conjugated steroids by the combined use of beta-glucuronidase preparations from helix pomatia and ampullaria: determination of urinary cortisol and its metabolites

21 January 2018

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Identification of bile acid precursors as endogenous ligands for the nuclear xenobiotic pregnane X receptor

21 January 2018

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Identification of intermediates in the bile acid synthetic pathway as ligands for the farnesoid X receptor

21 January 2018

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Loss of nuclear receptor SHP impairs but does not eliminate negative feedback regulation of bile acid synthesis

21 January 2018

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Redundant pathways for negative feedback regulation of bile acid production

21 January 2018

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An oxysterol signalling pathway mediated by the nuclear receptor LXR alpha

21 January 2018

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Structural requirements of ligands for the oxysterol liver X receptors LXRalpha and LXRbeta

21 January 2018

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Cholesterol and bile acid metabolism are impaired in mice lacking the nuclear oxysterol receptor LXR alpha

21 January 2018

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Differential regulation of rat and human CYP7A1 by the nuclear oxysterol receptor liver X receptor-alpha

21 January 2018

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Regulation of cholesterol 7alpha-hydroxylase gene (CYP7A1) transcription by the liver orphan receptor (LXRalpha).

21 January 2018

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Dietary cholesterol fails to stimulate the human cholesterol 7alpha-hydroxylase gene (CYP7A1) in transgenic mice

21 January 2018

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Oxysterol 7 alpha-hydroxylase activity by cholesterol 7 alpha-hydroxylase (CYP7A).

21 January 2018

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Differential expression of exons 1a and 1c in mRNAs for sterol regulatory element binding protein-1 in human and mouse organs and cultured cells

21 January 2018

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Abnormal high density lipoproteins in cerebrotendinous xanthomatosis

21 January 2018

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21 January 2018

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21 January 2018

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Mechanism of rifampicin and pregnane X receptor inhibition of human cholesterol 7 alpha-hydroxylase gene transcription.

21 January 2018

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Coordinated control of cholesterol catabolism to bile acids and of gluconeogenesis via a novel mechanism of transcription regulation linked to the fasted-to-fed cycle

21 January 2018

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Down-regulation of cholesterol 7alpha-hydroxylase (CYP7A1) gene expression by bile acids in primary rat hepatocytes is mediated by the c-Jun N-terminal kinase pathway

21 January 2018

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The negative effects of bile acids and tumor necrosis factor-alpha on the transcription of cholesterol 7alpha-hydroxylase gene (CYP7A1) converge to hepatic nuclear factor-4: a novel mechanism of feedback regulation of bile acid synthesis mediated by

21 January 2018

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Transcriptional regulation of the human sterol 12alpha-hydroxylase gene (CYP8B1): roles of heaptocyte nuclear factor 4alpha in mediating bile acid repression

21 January 2018

1 reference

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Regulation of human sterol 27-hydroxylase gene (CYP27A1) by bile acids and hepatocyte nuclear factor 4alpha (HNF4alpha).

21 January 2018

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Hepatocyte nuclear factor 4alpha (nuclear receptor 2A1) is essential for maintenance of hepatic gene expression and lipid homeostasis

21 January 2018

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Role of the hepatocyte nuclear factor 4alpha in control of the pregnane X receptor during fetal liver development

21 January 2018

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Cholic acid mediates negative feedback regulation of bile acid synthesis in mice

21 January 2018

1 reference

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10.1194/JLR.M400256-JLR200

21 January 2018

Identifiers

DOI

1 reference

2 September 2017

1 reference