(Q35212891)

7 August 2017

title

Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome (English)

1 reference

7 August 2017

Libin Xu

1

1 reference

7 August 2017

Wei Liu

2

1 reference

7 August 2017

Lowell G Sheflin

3

1 reference

7 August 2017

Steven J Fliesler

4

1 reference

7 August 2017

Ned A Porter

5

1 reference

7 August 2017

publication date

4 August 2011

1 reference

Journal of Lipid Research

7 August 2017

published in

1 reference

7 August 2017

volume

52

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7 August 2017

issue

10

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7 August 2017

page(s)

1810-1820

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Conversion of 7-dehydrocholesterol to 7-ketocholesterol is catalyzed by human cytochrome P450 7A1 and occurs by direct oxidation without an epoxide intermediate

7 August 2017

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28 July 2018

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28 July 2018

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Smith-Lemli-Opitz syndrome and autism spectrum disorder

28 July 2018

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Oxysterols: functional significance in fetal development and the maintenance of normal retinal function

28 July 2018

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Partial rescue of retinal function and sterol steady-state in a rat model of Smith-Lemli-Opitz syndrome

28 July 2018

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Light-induced exacerbation of retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome

28 July 2018

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Lipid hydroperoxide formation in the retina: correlation with retinal degeneration and light damage in a rat model of Smith-Lemli-Opitz syndrome

28 July 2018

1 reference

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Retinal degeneration in a rodent model of Smith-Lemli-Opitz syndrome: electrophysiologic, biochemical, and morphologic features

28 July 2018

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Cholesterol supplementation does not improve developmental progress in Smith-Lemli-Opitz syndrome.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Formation of biologically active oxysterols during ozonolysis of cholesterol present in lung surfactant

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Human malformation syndromes due to inborn errors of cholesterol synthesis

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Metabolism of 4 beta -hydroxycholesterol in humans.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Oxysterols in the circulation of patients with the Smith-Lemli-Opitz syndrome: abnormal levels of 24S- and 27-hydroxycholesterol.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Oxysterol biosynthetic enzymes

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Mutation analysis and description of sixteen RSH/Smith-Lemli-Opitz syndrome patients: polymerase chain reaction-based assays to simplify genotyping

28 July 2018

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Inborn errors of cholesterol biosynthesis

28 July 2018

1 reference

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The Smith-Lemli-Opitz syndrome

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Oxysterols: modulators of cholesterol metabolism and other processes

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

cDNA cloning of cholesterol 24-hydroxylase, a mediator of cholesterol homeostasis in the brain

28 July 2018

1 reference

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Photosensitivity associated with the Smith-Lemli-Opitz syndrome.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Cholesterol biosynthesis inhibited by BM15.766 induces holoprosencephaly in the rat.

28 July 2018

1 reference

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Clinical effects of cholesterol supplementation in six patients with the Smith-Lemli-Opitz syndrome (SLOS)

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Inhibition of 7-dehydrocholesterol reductase by the teratogen AY9944: a rat model for Smith-Lemli-Opitz syndrome.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Changes in serum sterols of rats treated with 7-dehydrocholesterol-delta 7-reductase inhibitors: comparison to levels in humans with Smith-Lemli-Opitz syndrome.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Defective cholesterol biosynthesis associated with the Smith-Lemli-Opitz syndrome

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Reproducing abnormal cholesterol biosynthesis as seen in the Smith-Lemli-Opitz syndrome by inhibiting the conversion of 7-dehydrocholesterol to cholesterol in rats

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Teratogenic Effect of an Inhibitor of Cholesterol Synthesis (AY 9944) in Rats: Correlation with Maternal Cholesterolemia

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

4alpha-Hydroxycholecalciferol and an attempted synthesis of its 4beta-hydroxy-epimer

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

The synthesis and biological activity of 25-hydroxy-26,27-dimethylvitamin D3 and 1,25-dihydroxy-26,27-dimethylvitamin D3: highly potent novel analogs of vitamin D3

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Cholesterol autoxidation 1981-1986.

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Biological activities of oxysterols

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Teratogenic action and embryo lethality of AY 9944R: Prevention by a hypercholesterolemia-provoking diet

28 July 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Defective cholesterol biosynthesis in Smith-Lemli-Opitz syndrome.

28 July 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

The near universal presence of autism spectrum disorders in children with Smith-Lemli-Opitz syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Abnormal cholesterol metabolism in the Smith-Lemli-Opitz syndrome: report of clinical and biochemical findings in four patients and treatment in one patient

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Cholesterol treatment forever? The first Scandinavian trial of cholesterol supplementation in the cholesterol-synthesis defect Smith-Lemli-Opitz syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Analysis of short-term behavioral effects of dietary cholesterol supplementation in Smith-Lemli-Opitz syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Rate constants for peroxidation of polyunsaturated fatty acids and sterols in solution and in liposomes

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Retinal degeneration in a rat model of Smith-Lemli-Opitz Syndrome: thinking beyond cholesterol deficiency

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Cytotoxic effects of oxysterols associated with human diseases: Induction of cell death (apoptosis and/or oncosis), oxidative and inflammatory activities, and phospholipidosis.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

AGENTS AFFECTING LIPID METABOLISM. XV. BIOCHEMICAL STUDIES WITH THE CHOLESTEROL BIOSYNTHESIS INHIBITOR AY-9944 IN YOUNG AND MATURE RATS.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Abnormal cholesterol biosynthesis as in Smith-Lemli-Opitz syndrome disrupts normal skeletal development in the rat.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Regional lipid composition in the rat brain

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Absence of ventral cell populations in the developing brain in a rat model of the Smith-Lemli-Opitz syndrome.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Antiepileptic drugs increase plasma levels of 4beta-hydroxycholesterol in humans: evidence for involvement of cytochrome p450 3A4.

21 January 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

Marked alteration of sterol metabolism and composition without compromising retinal development or function.

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Inherited disorders of cholesterol biosynthesis

26 September 2018

1 reference

https://api.crossref.org/works/10.1194%2FJLR.M018366

The oxysterols cholest-5-ene-3 beta,4 alpha-diol, cholest-5-ene-3 beta,4 beta-diol and cholestane-3 beta,5 alpha,6 alpha-triol are formed during in vitro oxidation of low density lipoprotein, and are present in human atherosclerotic plaques

21 January 2018

1 reference

https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pmc&linkname=pmc_refs_pubmed&retmode=json&id=3173002

Cholesta-5,7,9(11)-trien-3 beta-ol found in plasma of patients with Smith-Lemli-Opitz syndrome indicates formation of sterol hydroperoxide

8 December 2018

1 reference

12 December 2020

Treatment of Smith-Lemli-Opitz syndrome: results of a multicenter trial

1 reference

12 December 2020

Photochemical reactions and phototoxicity of sterols: novel self-perpetuating mechanisms for lipid photooxidation

1 reference

12 December 2020

7-Hydroperoxycholesterol and its products in oxidized low density lipoprotein and human atherosclerotic plaque

1 reference

12 December 2020

Oxysterols and atherosclerosis

1 reference

12 December 2020

An Inhibitor of Cholesterol Biosynthesis

1 reference

12 December 2020

Effects of cholesterol and simvastatin treatment in patients with Smith-Lemli-Opitz syndrome (SLOS)

1 reference

12 December 2020

Cytotoxicity of oxidized LDL to porcine aortic smooth muscle cells is associated with the oxysterols 7-ketocholesterol and 7-hydroxycholesterol

1 reference

12 December 2020

Doubly allylic hydroperoxide formed in the reaction between sterol 5,7-dienes and singlet oxygen

1 reference

12 December 2020

Endogenous 7-oxocholesterol is an enzymatic product: characterization of 7 alpha-hydroxycholesterol dehydrogenase activity of hamster liver microsomes

1 reference

12 December 2020

Suppression of 3-hydroxy-3-methylglutaryl coenzyme A reductase activity and inhibition of growth of human fibroblasts by 7-ketocholesterol

1 reference

12 December 2020

Identifiers

DOI

10.1194/JLR.M018366

1 reference

7 August 2017

1 reference

7 August 2017

1 reference