Published in J Biol Chem on April 09, 2003
Efavirenz for Patients With Mild Cognitive Impairment | NCT03706885
A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome. Nat Genet (2013) 2.59
Enzymatic reduction of oxysterols impairs LXR signaling in cultured cells and the livers of mice. Cell Metab (2007) 2.51
SIRT2 inhibition achieves neuroprotection by decreasing sterol biosynthesis. Proc Natl Acad Sci U S A (2010) 2.09
Brain cholesterol turnover required for geranylgeraniol production and learning in mice. Proc Natl Acad Sci U S A (2006) 1.77
Mutation of beta-glucosidase 2 causes glycolipid storage disease and impaired male fertility. J Clin Invest (2006) 1.66
Cholesterol 24-hydroxylase: an enzyme of cholesterol turnover in the brain. Annu Rev Biochem (2009) 1.61
Cholestenoic acids regulate motor neuron survival via liver X receptors. J Clin Invest (2014) 1.55
Neuronal expression and subcellular localization of cholesterol 24-hydroxylase in the mouse brain. J Comp Neurol (2008) 1.47
Inactivation of liver X receptor beta leads to adult-onset motor neuron degeneration in male mice. Proc Natl Acad Sci U S A (2005) 1.40
Gamma-secretase limits the inflammatory response through the processing of LRP1. Sci Signal (2008) 1.38
The use of the Dhcr7 knockout mouse to accurately determine the origin of fetal sterols. J Lipid Res (2006) 1.34
Specific loss of brain ABCA1 increases brain cholesterol uptake and influences neuronal structure and function. J Neurosci (2009) 1.31
Cholesterol in the retina: the best is yet to come. Prog Retin Eye Res (2014) 1.27
Central nervous system: cholesterol turnover, brain development and neurodegeneration. Biol Chem (2009) 1.25
ACAT1 gene ablation increases 24(S)-hydroxycholesterol content in the brain and ameliorates amyloid pathology in mice with AD. Proc Natl Acad Sci U S A (2010) 1.23
Cholesterol metabolism in Huntington disease. Nat Rev Neurol (2011) 1.14
Are side-chain oxidized oxysterols regulators also in vivo? J Lipid Res (2008) 1.12
Structural basis of drug binding to CYP46A1, an enzyme that controls cholesterol turnover in the brain. J Biol Chem (2010) 1.11
Liquid chromatography-mass spectrometry utilizing multi-stage fragmentation for the identification of oxysterols. J Lipid Res (2007) 1.11
The antifungal drug voriconazole is an efficient inhibitor of brain cholesterol 24S-hydroxylase in vitro and in vivo. J Lipid Res (2009) 1.08
Cholesterol: its regulation and role in central nervous system disorders. Cholesterol (2012) 1.08
The major brain cholesterol metabolite 24(S)-hydroxycholesterol is a potent allosteric modulator of N-methyl-D-aspartate receptors. J Neurosci (2013) 1.06
The effect of 24S-hydroxycholesterol on cholesterol homeostasis in neurons: quantitative changes to the cortical neuron proteome. J Proteome Res (2008) 1.04
Reduction of cholesterol synthesis in the mouse brain does not affect amyloid formation in Alzheimer's disease, but does extend lifespan. Proc Natl Acad Sci U S A (2009) 1.02
Cholesterol loss enhances TrkB signaling in hippocampal neurons aging in vitro. Mol Biol Cell (2008) 1.01
Differential expression and function of ABCG1 and ABCG4 during development and aging. J Lipid Res (2010) 1.01
Biphasic requirement for geranylgeraniol in hippocampal long-term potentiation. Proc Natl Acad Sci U S A (2008) 1.00
Antifungal Azoles: Structural Insights into Undesired Tight Binding to Cholesterol-Metabolizing CYP46A1. Mol Pharmacol (2013) 0.98
Analysis of bioactive oxysterols in newborn mouse brain by LC/MS. J Lipid Res (2012) 0.97
Cytochromes p450: roles in diseases. J Biol Chem (2013) 0.95
Cholesterol efflux is differentially regulated in neurons and astrocytes: implications for brain cholesterol homeostasis. Biochim Biophys Acta (2012) 0.92
In silico and intuitive predictions of CYP46A1 inhibition by marketed drugs with subsequent enzyme crystallization in complex with fluvoxamine. Mol Pharmacol (2012) 0.91
Pharmacologic stimulation of cytochrome P450 46A1 and cerebral cholesterol turnover in mice. J Biol Chem (2013) 0.91
Cholesterol as a causative factor in Alzheimer's disease: a debatable hypothesis. J Neurochem (2014) 0.90
On the regulatory role of side-chain hydroxylated oxysterols in the brain. Lessons from CYP27A1 transgenic and Cyp27a1(-/-) mice. J Lipid Res (2013) 0.89
Combined D2-/L2-hydroxyglutaric aciduria (SLC25A1 deficiency): clinical course and effects of citrate treatment. J Inherit Metab Dis (2014) 0.89
Survival of adult neurons lacking cholesterol synthesis in vivo. BMC Neurosci (2007) 0.88
Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model. Mol Cell Biol (2004) 0.88
Is it possible to improve memory function by upregulation of the cholesterol 24S-hydroxylase (CYP46A1) in the brain? PLoS One (2013) 0.88
Oxysterols in the brain of the cholesterol 24-hydroxylase knockout mouse. Biochem Biophys Res Commun (2014) 0.87
Function and comorbidities of apolipoprotein e in Alzheimer's disease. Int J Alzheimers Dis (2011) 0.87
Constitutive hippocampal cholesterol loss underlies poor cognition in old rodents. EMBO Mol Med (2014) 0.87
Flux analysis of cholesterol biosynthesis in vivo reveals multiple tissue and cell-type specific pathways. Elife (2015) 0.86
Cholesterol metabolism and homeostasis in the brain. Protein Cell (2015) 0.86
24S-hydroxycholesterol effects on lipid metabolism genes are modeled in traumatic brain injury. Brain Res (2010) 0.85
24(S)-Hydroxycholesterol as a Modulator of Neuronal Signaling and Survival. Neuroscientist (2015) 0.85
Histone deacetylase inhibition decreases cholesterol levels in neuronal cells by modulating key genes in cholesterol synthesis, uptake and efflux. PLoS One (2013) 0.84
Dietary intake of plant sterols stably increases plant sterol levels in the murine brain. J Lipid Res (2012) 0.84
Membrane lipid rafts and neurobiology: age-related changes in membrane lipids and loss of neuronal function. J Physiol (2015) 0.82
Interaction between positive allosteric modulators and trapping blockers of the NMDA receptor channel. Br J Pharmacol (2015) 0.82
Links between copper and cholesterol in Alzheimer's disease. Front Physiol (2013) 0.81
Selective reconstitution of liver cholesterol biosynthesis promotes lung maturation but does not prevent neonatal lethality in Dhcr7 null mice. BMC Dev Biol (2007) 0.81
Plasma 24S-hydroxycholesterol correlation with markers of Huntington disease progression. Neurobiol Dis (2013) 0.81
Linking lipids, Alzheimer's and LXRs? Nucl Recept Signal (2004) 0.81
Intracellular cholesterol homeostasis and amyloid precursor protein processing. Biochim Biophys Acta (2010) 0.81
Cortical injury increases cholesterol 24S hydroxylase (Cyp46) levels in the rat brain. J Neurotrauma (2008) 0.80
Neuronal cytochrome P450 activity and opioid analgesia: relevant sites and mechanisms. Brain Res (2015) 0.80
Cytochrome P450 and matrix metalloproteinase genetic modifiers of disease severity in Cerebral Cavernous Malformation type 1. Free Radic Biol Med (2016) 0.80
Retinal and nonocular abnormalities in Cyp27a1(-/-)Cyp46a1(-/-) mice with dysfunctional metabolism of cholesterol. Am J Pathol (2014) 0.79
CYP46A1, the rate-limiting enzyme for cholesterol degradation, is neuroprotective in Huntington's disease. Brain (2016) 0.79
Inhibiting cholesterol degradation induces neuronal sclerosis and epileptic activity in mouse hippocampus. Eur J Neurosci (2015) 0.78
Liver X receptors and cholesterol metabolism: role in ventral midbrain development and neurodegeneration. F1000Prime Rep (2015) 0.78
Endogenous 24S-hydroxycholesterol modulates NMDAR-mediated function in hippocampal slices. J Neurophysiol (2015) 0.78
Cholesterol-loaded nanoparticles ameliorate synaptic and cognitive function in Huntington's disease mice. EMBO Mol Med (2015) 0.77
Okadaic acid inhibits the trichostatin A-mediated increase of human CYP46A1 neuronal expression in a ERK1/2-Sp3-dependent pathway. J Lipid Res (2012) 0.77
Sulphatation does not appear to be a protective mechanism to prevent oxysterol accumulation in humans and mice. PLoS One (2013) 0.77
Mapping of the Allosteric Site in Cholesterol Hydroxylase CYP46A1 for Efavirenz, a Drug That Stimulates Enzyme Activity. J Biol Chem (2016) 0.77
25-Hydroxycholesterol regulates cholesterol homeostasis in the murine CATH.a neuronal cell line. Neurosci Lett (2013) 0.77
24S-hydroxycholesterol and 25-hydroxycholesterol differentially impact hippocampal neuronal survival following oxygen-glucose deprivation. PLoS One (2017) 0.75
Cytochrome P450 27A1 Deficiency and Regional Differences in Brain Sterol Metabolism Cause Preferential Cholestanol Accumulation in the Cerebellum. J Biol Chem (2017) 0.75
Suppression of cytochrome P450 reductase expression promotes astrocytosis in subventricular zone of adult mice. Neurosci Lett (2013) 0.75
Effects of bile acids on neurological function and disease. FASEB J (2016) 0.75
Phosphatidylcholine: Greasing the Cholesterol Transport Machinery. Lipid Insights (2016) 0.75
Neuronal cholesterol metabolism increases dendritic outgrowth and synaptic markers via a concerted action of GGTase-I and Trk. Sci Rep (2016) 0.75
Apolipoprotein D Overexpression Protects Against Kainate-Induced Neurotoxicity in Mice. Mol Neurobiol (2016) 0.75
In vitro cytochrome P450 46A1 (CYP46A1) activation by neuroactive compounds. J Biol Chem (2017) 0.75
A comprehensive classification system for lipids. J Lipid Res (2005) 6.52
LMSD: LIPID MAPS structure database. Nucleic Acids Res (2006) 6.21
Systemic delivery of genes to striated muscles using adeno-associated viral vectors. Nat Med (2004) 5.59
Transplanted bone marrow regenerates liver by cell fusion. Nature (2003) 5.06
Lipidomics reveals a remarkable diversity of lipids in human plasma. J Lipid Res (2010) 4.14
Clinical importance of the cytochromes P450. Lancet (2002) 3.77
AAV vector integration sites in mouse hepatocellular carcinoma. Science (2007) 3.73
Thematic review series: brain Lipids. Cholesterol metabolism in the central nervous system during early development and in the mature animal. J Lipid Res (2004) 3.30
Genetic evidence that the human CYP2R1 enzyme is a key vitamin D 25-hydroxylase. Proc Natl Acad Sci U S A (2004) 2.89
Reversal of defective lysosomal transport in NPC disease ameliorates liver dysfunction and neurodegeneration in the npc1-/- mouse. Proc Natl Acad Sci U S A (2009) 2.88
Discovery of a proneurogenic, neuroprotective chemical. Cell (2010) 2.78
Targeted transgene insertion into human chromosomes by adeno-associated virus vectors. Nat Biotechnol (2002) 2.73
Loss of nuclear receptor SHP impairs but does not eliminate negative feedback regulation of bile acid synthesis. Dev Cell (2002) 2.68
A suppressor screen in Mecp2 mutant mice implicates cholesterol metabolism in Rett syndrome. Nat Genet (2013) 2.59
Foamy virus vector integration sites in normal human cells. Proc Natl Acad Sci U S A (2006) 2.52
Enzymatic reduction of oxysterols impairs LXR signaling in cultured cells and the livers of mice. Cell Metab (2007) 2.51
Regulated accumulation of desmosterol integrates macrophage lipid metabolism and inflammatory responses. Cell (2012) 2.38
Adeno-associated virus vectors integrate at chromosome breakage sites. Nat Genet (2004) 2.27
A mouse macrophage lipidome. J Biol Chem (2010) 2.14
25-Hydroxycholesterol secreted by macrophages in response to Toll-like receptor activation suppresses immunoglobulin A production. Proc Natl Acad Sci U S A (2009) 2.14
Purification of nucleic acids by extraction with phenol:chloroform. CSH Protoc (2006) 2.04
Gene targeting in vivo by adeno-associated virus vectors. Nat Biotechnol (2006) 2.03
Mammalian wax biosynthesis. I. Identification of two fatty acyl-Coenzyme A reductases with different substrate specificities and tissue distributions. J Biol Chem (2004) 2.02
Engineering of human pluripotent stem cells by AAV-mediated gene targeting. Mol Ther (2010) 1.99
Genetic variations and treatments that affect the lifespan of the NPC1 mouse. J Lipid Res (2007) 1.99
De-orphanization of cytochrome P450 2R1: a microsomal vitamin D 25-hydroxilase. J Biol Chem (2003) 1.89
Cyclodextrin overcomes the transport defect in nearly every organ of NPC1 mice leading to excretion of sequestered cholesterol as bile acid. J Lipid Res (2009) 1.87
Large-scale analysis of adeno-associated virus vector integration sites in normal human cells. J Virol (2005) 1.85
Werner syndrome protein limits MYC-induced cellular senescence. Genes Dev (2003) 1.84
Cholic acid mediates negative feedback regulation of bile acid synthesis in mice. J Clin Invest (2002) 1.84
Control of cholesterol turnover in the mouse. J Biol Chem (2001) 1.78
Brain cholesterol turnover required for geranylgeraniol production and learning in mice. Proc Natl Acad Sci U S A (2006) 1.77
Gene targeting in stem cells from individuals with osteogenesis imperfecta. Science (2004) 1.75
Liver X receptor activation enhances cholesterol loss from the brain, decreases neuroinflammation, and increases survival of the NPC1 mouse. J Neurosci (2007) 1.71
Improved foamy virus vectors with minimal viral sequences. Mol Ther (2002) 1.71
AAV-mediated gene targeting methods for human cells. Nat Protoc (2011) 1.70
Mutation of beta-glucosidase 2 causes glycolipid storage disease and impaired male fertility. J Clin Invest (2006) 1.66
Extraction and analysis of sterols in biological matrices by high performance liquid chromatography electrospray ionization mass spectrometry. Methods Enzymol (2007) 1.63
Human osteoblast-like cells express predominantly steroid 5alpha-reductase type 1. J Clin Endocrinol Metab (2002) 1.62
Cholesterol 24-hydroxylase: an enzyme of cholesterol turnover in the brain. Annu Rev Biochem (2009) 1.61
Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life. Pediatr Res (2010) 1.59
Successful treatment of canine leukocyte adhesion deficiency by foamy virus vectors. Nat Med (2007) 1.58
Genetic defects in bile acid conjugation cause fat-soluble vitamin deficiency. Gastroenterology (2013) 1.57
Lysosomal unesterified cholesterol content correlates with liver cell death in murine Niemann-Pick type C disease. J Lipid Res (2007) 1.54
Frequent endonuclease cleavage at off-target locations in vivo. Mol Ther (2010) 1.52
Human gene targeting by adeno-associated virus vectors is enhanced by DNA double-strand breaks. Mol Cell Biol (2003) 1.51
Cholesterol accumulation and liver cell death in mice with Niemann-Pick type C disease. Hepatology (2005) 1.48
A comprehensive method for extraction and quantitative analysis of sterols and secosteroids from human plasma. J Lipid Res (2012) 1.48
Neuronal expression and subcellular localization of cholesterol 24-hydroxylase in the mouse brain. J Comp Neurol (2008) 1.47
Chromosomal effects of adeno-associated virus vector integration. Nat Genet (2002) 1.47
Subcellular organelle lipidomics in TLR-4-activated macrophages. J Lipid Res (2010) 1.46
Quantitation of two pathways for cholesterol excretion from the brain in normal mice and mice with neurodegeneration. J Lipid Res (2003) 1.45
Oxysterol gradient generation by lymphoid stromal cells guides activated B cell movement during humoral responses. Immunity (2012) 1.44
Cell cycle requirements for transduction by foamy virus vectors compared to those of oncovirus and lentivirus vectors. J Virol (2004) 1.44
Delineation of molecular changes in intrahepatic cholesterol metabolism resulting from diminished cholesterol absorption. J Lipid Res (2005) 1.42
Gene targeting with viral vectors. Mol Ther (2005) 1.42
Molecular, anatomical, and biochemical events associated with neurodegeneration in mice with Niemann-Pick type C disease. J Neuropathol Exp Neurol (2005) 1.39
Mammalian wax biosynthesis. II. Expression cloning of wax synthase cDNAs encoding a member of the acyltransferase enzyme family. J Biol Chem (2004) 1.38
Isoform- and cell cycle-dependent substrate degradation by the Fbw7 ubiquitin ligase. J Cell Biol (2008) 1.35
Trisomy correction in Down syndrome induced pluripotent stem cells. Cell Stem Cell (2012) 1.35
Unesterified cholesterol accumulation in late endosomes/lysosomes causes neurodegeneration and is prevented by driving cholesterol export from this compartment. J Neurosci (2011) 1.33
Transduction of human NOD/SCID-repopulating cells with both lymphoid and myeloid potential by foamy virus vectors. Proc Natl Acad Sci U S A (2002) 1.32
Gene targeting of mutant COL1A2 alleles in mesenchymal stem cells from individuals with osteogenesis imperfecta. Mol Ther (2007) 1.31
Induction of hepatocellular carcinoma by in vivo gene targeting. Proc Natl Acad Sci U S A (2012) 1.31
ACAT2 deficiency limits cholesterol absorption in the cholesterol-fed mouse: impact on hepatic cholesterol homeostasis. Hepatology (2004) 1.29
Mutations in PPIB (cyclophilin B) delay type I procollagen chain association and result in perinatal lethal to moderate osteogenesis imperfecta phenotypes. Hum Mol Genet (2011) 1.23
Ontogenesis and regulation of cholesterol metabolism in the central nervous system of the mouse. Brain Res Dev Brain Res (2003) 1.22
Expression of the androgen receptor and 5 alpha-reductase type 2 in the developing human fetal penis and urethra. Cell Tissue Res (2001) 1.21
Receptor-mediated and bulk-phase endocytosis cause macrophage and cholesterol accumulation in Niemann-Pick C disease. J Lipid Res (2007) 1.21
Adeno-associated virus vector integration. Curr Opin Mol Ther (2009) 1.20
HLA engineering of human pluripotent stem cells. Mol Ther (2013) 1.19
Globin phenotype of erythroid cells derived from human induced pluripotent stem cells. Blood (2010) 1.19
Foamy-virus-mediated gene transfer to canine repopulating cells. Blood (2006) 1.18
A rapid and quantitative assay for measuring neighboring gene activation by vector proviruses. Mol Ther (2008) 1.15
ABCA1 plays no role in the centripetal movement of cholesterol from peripheral tissues to the liver and intestine in the mouse. J Lipid Res (2009) 1.14
CYP7B1: one cytochrome P450, two human genetic diseases, and multiple physiological functions. J Biol Chem (2009) 1.13
Gene transfer with foamy virus vectors. Methods Enzymol (2002) 1.13
Reduced genotoxicity of avian sarcoma leukosis virus vectors in rhesus long-term repopulating cells compared to standard murine retrovirus vectors. Mol Ther (2008) 1.12
Calcium-phosphate-mediated Transfection of Eukaryotic Cells with Plasmid DNAs. CSH Protoc (2006) 1.11
Quantitative role of LAL, NPC2, and NPC1 in lysosomal cholesterol processing defined by genetic and pharmacological manipulations. J Lipid Res (2011) 1.09