The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
about
An oxysterol biomarker for 7-dehydrocholesterol oxidation in cell/mouse models for Smith-Lemli-Opitz syndromeSteroid production and excretion by the pregnant mouse, particularly in relation to pregnancies with fetuses deficient in Delta7-sterol reductase (Dhcr7), the enzyme associated with Smith-Lemli-Opitz syndromeNanostructure-initiator mass spectrometry (NIMS) imaging of brain cholesterol metabolites in Smith-Lemli-Opitz syndromeCurrent trends in oxysterol researchCholesterol metabolism is required for intracellular hedgehog signal transduction in vivoFlux analysis of cholesterol biosynthesis in vivo reveals multiple tissue and cell-type specific pathwaysBiosynthesis of cholesterol and other sterolsComparison of the liquid-ordered bilayer phases containing cholesterol or 7-dehydrocholesterol in modeling Smith-Lemli-Opitz syndromeSelective reconstitution of liver cholesterol biosynthesis promotes lung maturation but does not prevent neonatal lethality in Dhcr7 null mice.Significant contributions of the extraembryonic membranes and maternal genotype to the placental pathology in heterozygous Nsdhl deficient female embryosActivation of Rho GTPases in Smith-Lemli-Opitz syndrome: pathophysiological and clinical implications.24S,25-Epoxycholesterol in mouse and rat brain.Biochemical and Physiological Improvement in a Mouse Model of Smith-Lemli-Opitz Syndrome (SLOS) Following Gene Transfer with AAV Vectors.Smith-Lemli-Opitz syndrome and inborn errors of cholesterol synthesis: summary of the 2007 SLO/RSH Foundation scientific conference sponsored by the National Institutes of Health.Desmosterol in brain is elevated because DHCR24 needs REST for Robust Expression but REST is poorly expressed.RNA-Seq analysis uncovers transcriptomic variations between morphologically similar in vivo- and in vitro-derived bovine blastocystsMalformation syndromes caused by disorders of cholesterol synthesis.Pathogenesis, Epidemiology, Diagnosis and Clinical Aspects of Smith-Lemli-Opitz Syndrome.Analysis of hedgehog signaling in cerebellar granule cell precursors in a conditional Nsdhl allele demonstrates an essential role for cholesterol in postnatal CNS development.A compendium of expression patterns of cholesterol biosynthetic enzymes in the mouse embryo.Suboptimal culture conditions induce more deviations in gene expression in male than female bovine blastocysts.Mitochondrial Citrate Transporter-dependent Metabolic Signature in the 22q11.2 Deletion SyndromeAnalysis of bioactive oxysterols in newborn mouse brain by LC/MS.Review: Transport of maternal cholesterol to the fetal circulation.Abnormal barrier function in the pathogenesis of ichthyosis: therapeutic implications for lipid metabolic disorders.Altered cholesterol biosynthesis causes precocious neurogenesis in the developing mouse forebrain.Prolactin receptor-associated protein/17beta-hydroxysteroid dehydrogenase type 7 gene (Hsd17b7) plays a crucial role in embryonic development and fetal survival.7-dehydrocholesterol efficiently supports Ret signaling in a mouse model of Smith-Opitz-Lemli syndrome.Transport of maternal cholesterol to the fetus is affected by maternal plasma cholesterol concentrations in the golden Syrian hamsterInability to fully suppress sterol synthesis rates with exogenous sterol in embryonic and extraembyronic fetal tissuesDevelopmental expression pattern of the cholesterogenic enzyme NSDHL and negative selection of NSDHL-deficient cells in the heterozygous Bpa(1H)/+ mouse.A highly sensitive method for analysis of 7-dehydrocholesterol for the study of Smith-Lemli-Opitz syndromeDisrupting Hepatocyte Cyp51 from Cholesterol Synthesis Leads to Progressive Liver Injury in the Developing Mouse and Decreases RORC Signalling.From cholesterogenesis to steroidogenesis: role of riboflavin and flavoenzymes in the biosynthesis of vitamin D.Analysis by liquid chromatography-mass spectrometry of sterols and oxysterols in brain of the newborn Dhcr7(Δ3-5/T93M) mouse: a model of Smith-Lemli-Opitz syndrome.Defects in cholesterol synthesis genes in mouse and in humans: lessons for drug development and safer treatments.Disorders of sterol synthesis: beyond Smith-Lemli-Opitz syndrome.Smith-Lemli-Opitz syndrome: phenotype, natural history, and epidemiology.The role of maternal-fetal cholesterol transport in early fetal life: current insights.Pathogenesis of the cutaneous phenotype in inherited disorders of cholesterol metabolism: Therapeutic implications for topical treatment of these disorders.
P2860
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P2860
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
description
2006 nî lūn-bûn
@nan
2006 թուականի Մայիսին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի մայիսին հրատարակված գիտական հոդված
@hy
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
name
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@ast
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@en
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@nl
type
label
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@ast
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@en
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@nl
prefLabel
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@ast
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@en
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@nl
P2093
P2860
P1476
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols.
@en
P2093
Guorong Xu
Hongwei Yu
Quan Shang
Shailendra B Patel
P2860
P304
P356
10.1194/JLR.M600141-JLR200
P577
2006-05-01T00:00:00Z