7-Dehydrocholesterol-derived oxysterols and retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome.

No abstract is available for this paper.

• Publication year

  2012

• Venue

  Biochimica et Biophysica Acta

• Publication date

  2012-06-01

• Fields of study

  Biology, Medicine, Chemistry

• Identifiers
  DOI 10.1016/j.bbalip.2012.03.001PMID 22425966PMCID PMC3340457
• External record

  Open on Semantic Scholar

• Source metadata

  Semantic Scholar, PubMed

• No claims are published for this paper.

• No concepts are published for this paper.

• DHCEO accumulation is a critical mediator of pathophysiology in a Smith-Lemli-Opitz syndrome model.

  2012cited by this paper
• Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome[S]

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  2011cited by this paper
• Conversion of 7-Dehydrocholesterol to 7-Ketocholesterol Is Catalyzed by Human Cytochrome P450 7A1 and Occurs by Direct Oxidation without an Epoxide Intermediate

  2011cited by this paper
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  2010cited by this paper
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  2010cited by this paper
• Mitochondrial DNA damage induced by 7-ketocholesterol in human retinal pigment epithelial cells in vitro.

  2010cited by this paper
• The ins and outs of cholesterol in the vertebrate retina

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• Biological activities of 7-dehydrocholesterol-derived oxysterols: implications for Smith-Lemli-Opitz syndrome[S]

  2010cited by this paper
• Cholesterol oxidation in the retina: implications of 7KCh formation in chronic inflammation and age-related macular degeneration

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• Analysis of short‐term behavioral effects of dietary cholesterol supplementation in Smith–Lemli–Opitz syndrome

  2010cited by this paper
• Rate constants for peroxidation of polyunsaturated fatty acids and sterols in solution and in liposomes.

  2009cited by this paper
• Photodamage Generates 7‐keto‐ and 7‐hydroxycholesterol in the Rat Retina via a Free Radical‐mediated Mechanism

  2009cited by this paper
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  2009cited by this paper
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• Naturally occurring animal models with outer retina phenotypes.

  2009cited by this paper
• Effects of Dietary Cholesterol and Simvastatin on Cholesterol Synthesis in Smith-Lemli-Opitz Syndrome

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  2008cited by this paper
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  2007cited by this paper
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  2007cited by this paper
• Redox regulation of 7-ketocholesterol-induced apoptosis by beta-carotene in human macrophages.

  2007cited by this paper
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  2006cited by this paper
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• Light-induced exacerbation of retinal degeneration in a rat model of Smith-Lemli-Opitz syndrome.

  2006cited by this paper
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  2004cited by this paper
• Retinal degeneration in a rodent model of Smith-Lemli-Opitz syndrome: electrophysiologic, biochemical, and morphologic features.

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  2003cited by this paper
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• 7-Dehydrocholesterol-dependent proteolysis of HMG-CoA reductase suppresses sterol biosynthesis in a mouse model of Smith-Lemli-Opitz/RSH syndrome.

  2001cited by this paper
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• Retinal Structure and Function in an Animal Model that Replicates the Biochemical Hallmarks of Desmosterolosis

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• Cholesterol synthesis in the vertebrate retina: Effects of U18666A on rat retinal structure, photoreceptor membrane assembly, and sterol metabolism and composition

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• The Smith-Lemli-Opitz syndrome

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• Pleiotropic effects of statins

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• Oxysterols: modulators of cholesterol metabolism and other processes.

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• Cyp7b, a novel brain cytochrome P450, catalyzes the synthesis of neurosteroids 7α-hydroxy dehydroepiandrosterone and 7α-hydroxy pregnenolone

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  1996cited by this paper
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• Smith‐Lemli‐Opitz Syndrome: Neuropathological and Ophthalmological Observations

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• Exploring Recent Developments in the Manifestation, Diagnosis, and Treatment of Patients with Smith–Lemli–Opitz Syndrome: From Molecular Pathways to Clinical Innovations

  2025cites this paper
• Hydroxyzine Effects on Post-Lanosterol Biosynthesis in Smith–Lemli–Opitz Syndrome (SLOS) Models

  2025cites this paper
• "The role of cholesterol biosynthesis and metabolism causing medical complexity in patients with Smith-Lemli-Opitz Syndrome (SLOS)".

  2025cites this paper
• Radiation-induced ferroptosis via liposomal delivery of 7-Dehydrocholesterol

  2025cites this paper
• Inhibition of 7-dehydrocholesterol reductase prevents hepatic ferroptosis under an active state of sterol synthesis

  2024cites this paper
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• The drug release of PLGA-based nanoparticles and their application in treatment of gastrointestinal cancers

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• Cholic acid increases plasma cholesterol in Smith-Lemli-Opitz syndrome: A pilot study

  2023cites this paper
• Smith-Lemli-Opitz syndrome: A pathophysiological manifestation of the Bloch hypothesis

  2023cites this paper
• 7-Dehydrocholesterol Encapsulated Polymeric Nanoparticles As a Radiation-Responsive Sensitizer for Enhancing Radiation Therapy.

  2022cites this paper
• Prescription Medications Alter Neuronal and Glial Cholesterol Synthesis.

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• Inhibition of lysosomal phospholipase A2 predicts drug-induced phospholipidosis

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• Oxysterols and retinal degeneration

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• Transcriptomic Changes Associated with Loss of Cell Viability Induced by Oxysterol Treatment of a Retinal Photoreceptor-Derived Cell Line: An In Vitro Model of Smith–Lemli–Opitz Syndrome

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• Cholesterol homeostasis in the vertebrate retina: Biology and pathobiology.

  2020cites this paper
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• Cholesterol Biosynthesis in the Nervous System with an Emphasis on Desmosterolosis

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• 7-Ketocholesterol in disease and aging

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• 7-Ketocholesterol disturbs RPE cells phagocytosis of the outer segment of photoreceptor and induces inflammation through ERK signaling pathway.

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  2018cites this paper
• Prevention of Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome

  2018cites this paper
• Compromised phagosome maturation underlies RPE pathology in cell culture and whole animal models of Smith-Lemli-Opitz Syndrome

  2018cites this paper
• Oxysterols and Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome: Implications for an Improved Therapeutic Intervention

  2018cites this paper
• Sterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patients.

  2017cites this paper
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  2017cites this paper
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• The Effect of Small Molecules on Sterol Homeostasis: Measuring 7-Dehydrocholesterol in Dhcr7-Deficient Neuro2a Cells and Human Fibroblasts.

  2016cites this paper
• 7DHC-induced changes of Kv1.3 operation contributes to modified T cell function in Smith-Lemli-Opitz syndrome

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• Reduced cholesterol levels impair Smoothened activation in Smith–Lemli–Opitz syndrome

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• On the Role of Membrane Structural Defects in Smith-Lemli-Opitz Syndrome

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• Ion-current-based Proteomic Profiling of the Retina in a Rat Model of Smith-Lemli-Opitz Syndrome*

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  2012cites this paper
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  2012cites this paper
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