DHCEO accumulation is a critical mediator of pathophysiology in a Smith-Lemli-Opitz syndrome model
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Lipid 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 syndromeFam57b (family with sequence similarity 57, member B), a novel peroxisome proliferator-activated receptor γ target gene that regulates adipogenesis through ceramide synthesisThe Effect of Small Molecules on Sterol Homeostasis: Measuring 7-Dehydrocholesterol in Dhcr7-Deficient Neuro2a Cells and Human FibroblastsAntioxidant supplementation ameliorates molecular deficits in Smith-Lemli-Opitz syndromeProbing lipid-protein adduction with alkynyl surrogates: application to Smith-Lemli-Opitz syndrome.Free 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.Metabolism of oxysterols derived from nonenzymatic oxidation of 7-dehydrocholesterol in cells.Altered cholesterol biosynthesis causes precocious neurogenesis in the developing mouse forebrain.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.A highly sensitive method for analysis of 7-dehydrocholesterol for the study of Smith-Lemli-Opitz syndromeReactivities and products of free radical oxidation of cholestadienols.Smith-Lemli-Opitz syndrome: phenotype, natural history, and epidemiology.Oxidative stress, serotonergic changes and decreased ultrasonic vocalizations in a mouse model of Smith-Lemli-Opitz syndrome.Differential cytotoxic effects of 7-dehydrocholesterol-derived oxysterols on cultured retina-derived cells: Dependence on sterol structure, cell type, and density.Hepatic isoprenoid metabolism in a rat model of Smith-Lemli-Opitz Syndrome.Antioxidants: The Missing Key to Improved Therapeutic Intervention in Smith-Lemli-Opitz Syndrome?Birthday of a syndrome: 50 years anniversary of Smith-Lemli-Opitz Syndrome.Lipid metabolism fattens up hedgehog signaling.Prevention of Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome.Assessment of altered lipid homeostasis by HILIC-ion mobility-mass spectrometry-based lipidomics.Oxysterols and Retinal Degeneration in a Rat Model of Smith-Lemli-Opitz Syndrome: Implications for an Improved Therapeutic Intervention
P2860
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P2860
DHCEO accumulation is a critical mediator of pathophysiology in a Smith-Lemli-Opitz syndrome model
description
2011 nî lūn-bûn
@nan
2011年の論文
@ja
2011年論文
@yue
2011年論文
@zh-hant
2011年論文
@zh-hk
2011年論文
@zh-mo
2011年論文
@zh-tw
2011年论文
@wuu
2011年论文
@zh
2011年论文
@zh-cn
name
DHCEO accumulation is a critic ...... ith-Lemli-Opitz syndrome model
@en
type
label
DHCEO accumulation is a critic ...... ith-Lemli-Opitz syndrome model
@en
prefLabel
DHCEO accumulation is a critic ...... ith-Lemli-Opitz syndrome model
@en
P2093
P2860
P1476
DHCEO accumulation is a critic ...... ith-Lemli-Opitz syndrome model
@en
P2093
Aaron B Bowman
Jennifer Da
Karoly Mirnics
Ned A Porter
Zeljka Korade
P2860
P304
P356
10.1016/J.NBD.2011.12.011
P577
2011-12-11T00:00:00Z