Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease.
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NPC2 facilitates bidirectional transfer of cholesterol between NPC1 and lipid bilayers, a step in cholesterol egress from lysosomesReceptor-mediated endocytosis: insights from the lipoprotein receptor systemThe LDL receptorStructure of N-Terminal Domain of NPC1 Reveals Distinct Subdomains for Binding and Transfer of CholesterolThe Saccharomyces cerevisiae YLL012/YEH1, YLR020/YEH2, and TGL1 genes encode a novel family of membrane-anchored lipases that are required for steryl ester hydrolysis.A lysosome-centered view of nutrient homeostasisNovel treatment options for lysosomal acid lipase deficiency: critical appraisal of sebelipase alfaCharacterization of the Niemann-Pick C pathway in alveolar type II cells and lamellar bodies of the lungGenomics and proteomics of vertebrate cholesterol ester lipase (LIPA) and cholesterol 25-hydroxylase (CH25H)Comparative studies of mammalian acid lipases: Evidence for a new gene family in mouse and rat (Lipo)The Niemann-Pick C1 and caveolin-1 proteins interact to modulate efflux of low density lipoprotein-derived cholesterol from late endocytic compartments.Chemical screen to reduce sterol accumulation in Niemann-Pick C disease cells identifies novel lysosomal acid lipase inhibitors.Metabolism of cationized lipoproteins by human fibroblasts. Biochemical and morphologic correlationsDevelopment of a novel, cell-based chemical screen to identify inhibitors of intraphagosomal lipolysis in macrophages.Cholesterol pathways affected by small molecules that decrease sterol levels in Niemann-Pick type C mutant cells.Carboxylesterases: Dual roles in lipid and pesticide metabolism.Thyroid hormones control lysosomal enzyme activities in liver and skeletal muscle.Stimulation with a monoclonal antibody (mAb4E4) of scavenger receptor-mediated uptake of chemically modified low density lipoproteins by THP-1-derived macrophages enhances foam cell generation.The role of mannosylated enzyme and the mannose receptor in enzyme replacement therapyHepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient miceReversible accumulation of cholesteryl esters in macrophages incubated with acetylated lipoproteinsLoss of stearoyl-CoA desaturase activity leads to free cholesterol synthesis through increased Xbp-1 splicing.Lysosomal acid lipase: at the crossroads of normal and atherogenic cholesterol metabolismModified lipoprotein-derived lipid particles accumulate in human stenotic aortic valves.Comparison of regulation of 3-hydroxy-3-methylglutaryl coenzyme A reductase in hepatoma cells grown in vivo and in vitroDegradation of cationized low density lipoprotein and regulation of cholesterol metabolism in homozygous familial hypercholesterolemia fibroblasts.Deletion of sterol O-acyltransferase 2 (SOAT2) function in mice deficient in lysosomal acid lipase (LAL) dramatically reduces esterified cholesterol sequestration in the small intestine and liver.Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.The metabolic serine hydrolases and their functions in mammalian physiology and disease.Suppression of apolipoprotein B production during treatment of cholesteryl ester storage disease with lovastatin. Implications for regulation of apolipoprotein B synthesisCholesterol accumulation in tissues of the Niemann-pick type C mouse is determined by the rate of lipoprotein-cholesterol uptake through the coated-pit pathway in each organ.Regulation of low density lipoprotein receptor activity in freshly isolated human lymphocytes.PRD125, a potent and selective inhibitor of sterol O-acyltransferase 2 markedly reduces hepatic cholesteryl ester accumulation and improves liver function in lysosomal acid lipase-deficient micePinocytosis and intracellular degradation of exogenous protein: modulation by amino acidsHigh-capacity selective uptake of cholesteryl ester from native LDL during macrophage foam cell formation.Isolation and characterization of Chinese hamster ovary cell mutants defective in intracellular low density lipoprotein-cholesterol traffickingBinding, internalization, and hydrolysis of low density lipoprotein in long-term lymphoid cell lines from a normal subject and a patient with homozygous familial hypercholesterolemia.Metabolism of native and of lactosylated human low density lipoprotein: evidence for two pathways for catabolism of exogenous proteins in rat hepatocytes.Wolman disease/cholesteryl ester storage disease: efficacy of plant-produced human lysosomal acid lipase in mice.From Loci to Biology: Functional Genomics of Genome-Wide Association for Coronary Disease
P2860
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P2860
Role of lysosomal acid lipase in the metabolism of plasma low density lipoprotein. Observations in cultured fibroblasts from a patient with cholesteryl ester storage disease.
description
1975 nî lūn-bûn
@nan
1975年の論文
@ja
1975年学术文章
@wuu
1975年学术文章
@zh-cn
1975年学术文章
@zh-hans
1975年学术文章
@zh-my
1975年学术文章
@zh-sg
1975年學術文章
@yue
1975年學術文章
@zh
1975年學術文章
@zh-hant
name
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@en
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@nl
type
label
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@en
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@nl
prefLabel
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@en
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@nl
P2093
P1476
Role of lysosomal acid lipase ...... esteryl ester storage disease.
@en
P2093
A L Beaudet
J L Goldstein
P304
P407
P577
1975-11-01T00:00:00Z