Biological activities of 7-dehydrocholesterol-derived oxysterols: implications for Smith-Lemli-Opitz syndrome
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An oxysterol biomarker for 7-dehydrocholesterol oxidation in cell/mouse models for Smith-Lemli-Opitz syndromeLipid biomarkers of oxidative stress in a genetic mouse model of Smith-Lemli-Opitz syndrome7-Dehydrocholesterol-derived oxysterols and retinal degeneration in a rat model of Smith-Lemli-Opitz syndromeSterols and oxysterols in plasma from Smith-Lemli-Opitz syndrome patientsThe Effect of Small Molecules on Sterol Homeostasis: Measuring 7-Dehydrocholesterol in Dhcr7-Deficient Neuro2a Cells and Human FibroblastsIon-current-based proteomic profiling of the retina in a rat model of Smith-Lemli-Opitz syndrome.Feeding impairments associated with plasma sterols in Smith-Lemli-Opitz syndrome.The ins and outs of cholesterol in the vertebrate retina.Antioxidant supplementation ameliorates molecular deficits in Smith-Lemli-Opitz syndromeHypomorphic temperature-sensitive alleles of NSDHL cause CK syndromeMalformation syndromes caused by disorders of cholesterol synthesis.Lovastatin induces multiple stress pathways including LKB1/AMPK activation that regulate its cytotoxic effects in squamous cell carcinoma cells.Elevated Autophagy and Mitochondrial Dysfunction in the Smith-Lemli-Opitz Syndrome.Probing lipid-protein adduction with alkynyl surrogates: application to Smith-Lemli-Opitz syndrome.Pathogenesis, Epidemiology, Diagnosis and Clinical Aspects of Smith-Lemli-Opitz Syndrome.Novel oxysterols observed in tissues and fluids of AY9944-treated rats: a model for Smith-Lemli-Opitz syndrome.Profiling and Imaging Ion Mobility-Mass Spectrometry Analysis of Cholesterol and 7-Dehydrocholesterol in Cells Via Sputtered Silver MALDIFree radical oxidation of cholesterol and its precursors: Implications in cholesterol biosynthesis disorders.Inhibitors of 7-Dehydrocholesterol Reductase: Screening of a Collection of Pharmacologically Active Compounds in Neuro2a Cells.Tunneling in tocopherol-mediated peroxidation of 7-dehydrocholesterol.No evidence for mevalonate shunting in moderately affected children with Smith-Lemli-Opitz syndromeOxidation of 7-dehydrocholesterol and desmosterol by human cytochrome P450 46A1.Assays of plasma dehydrocholesteryl esters and oxysterols from Smith-Lemli-Opitz syndrome patientsMutational spectrum of smith-lemli-opitz syndrome patients in hungary.Metabolism of oxysterols derived from nonenzymatic oxidation of 7-dehydrocholesterol in cells.Altered cholesterol biosynthesis causes precocious neurogenesis in the developing mouse forebrain.DHCEO accumulation is a critical mediator of pathophysiology in a Smith-Lemli-Opitz syndrome modelA perspective on free radical autoxidation: the physical organic chemistry of polyunsaturated fatty acid and sterol peroxidation.Challenging behavior in Smith-Lemli-Opitz syndrome: initial test of biobehavioral influences.Identification of Environmental Quaternary Ammonium Compounds as Direct Inhibitors of Cholesterol Biosynthesis.Endogenous B-ring oxysterols inhibit the Hedgehog component Smoothened in a manner distinct from cyclopamine or side-chain oxysterols.7-dehydrocholesterol efficiently supports Ret signaling in a mouse model of Smith-Opitz-Lemli syndrome.A highly sensitive method for analysis of 7-dehydrocholesterol for the study of Smith-Lemli-Opitz syndromeProbes for protein adduction in cholesterol biosynthesis disorders: Alkynyl lanosterol as a viable sterol precursor.Reactivities and products of free radical oxidation of cholestadienols.Treatment of Smith-Lemli-Opitz syndrome and other sterol disordersProtein lipid modifications--More than just a greasy ballast.Conversion of 7-dehydrocholesterol to 7-ketocholesterol is catalyzed by human cytochrome P450 7A1 and occurs by direct oxidation without an epoxide intermediate.Oxidative stress, serotonergic changes and decreased ultrasonic vocalizations in a mouse model of Smith-Lemli-Opitz syndrome.Brain Cholesterol Metabolism and Its Defects: Linkage to Neurodegenerative Diseases and Synaptic Dysfunction.
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P2860
Biological activities of 7-dehydrocholesterol-derived oxysterols: implications for Smith-Lemli-Opitz syndrome
description
2010 nî lūn-bûn
@nan
2010 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2010 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2010年の論文
@ja
2010年論文
@yue
2010年論文
@zh-hant
2010年論文
@zh-hk
2010年論文
@zh-mo
2010年論文
@zh-tw
2010年论文
@wuu
name
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@ast
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@en
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@nl
type
label
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@ast
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@en
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@nl
prefLabel
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@ast
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@en
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@nl
P2093
P2860
P356
P1476
Biological activities of 7-deh ...... for Smith-Lemli-Opitz syndrome
@en
P2093
Ned A Porter
Richard Shelton
Zeljka Korade
P2860
P304
P356
10.1194/JLR.M009365
P577
2010-08-11T00:00:00Z