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Novel treatment options for lysosomal acid lipase deficiency: critical appraisal of sebelipase alfaPrenatal diagnosis of Wolman's disease.Hepatic entrapment of esterified cholesterol drives continual expansion of whole body sterol pool in lysosomal acid lipase-deficient miceSebelipase alfa over 52 weeks reduces serum transaminases, liver volume and improves serum lipids in patients with lysosomal acid lipase deficiency.Lysosomal acid lipase: at the crossroads of normal and atherogenic cholesterol metabolismReceptor-dependent hydrolysis of cholesteryl esters contained in plasma low density lipoprotein.Cholesteryl ester storage disease and Wolman disease: phenotypic variants of lysosomal acid cholesteryl ester hydrolase deficiency.Separation and characterization of the acid lipase and neutral esterases from human liverPRD125, 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 miceCharacterization of lysosomal acid lipase by site-directed mutagenesis and heterologous expression.The cytosolic adaptor AP-1A is essential for the trafficking and function of Niemann-Pick type C proteinsLysosomal acid lipase regulates VLDL synthesis and insulin sensitivity in miceNiemann-Pick type C disease: molecular mechanisms and potential therapeutic approaches.Breaking fat: The regulation and mechanisms of lipophagy.Effects of calcium antagonists on atherogenesis.Managing Cardiovascular Risk in Lysosomal Acid Lipase Deficiency.Characteristics and postnatal development of the acid lipase activity of the rat small intestine.Lysosomal acid lipase deficiency: a form of non-obese fatty liver disease (NOFLD).Possible physiological roles of carboxylic ester hydrolases.Studies on cholesterol ester formation and hydrolysis in liver disease: a selective review.Wolman disease and its treatment.Acid lipase cross-reacting material in Wolman disease and cholesterol ester storage diseaseContinuous transport of a small fraction of plasma membrane cholesterol to endoplasmic reticulum regulates total cellular cholesterol.A Carbon Nanotube Optical Reporter Maps Endolysosomal Lipid Flux.Impact of loss of SOAT2 function on disease progression in the lysosomal acid lipase-deficient mouse.Cytosolic lipolysis and lipophagy: two sides of the same coin.Lysosomal acid lipase regulates fatty acid channeling in brown adipose tissue to maintain thermogenesis.Association of LIPA Gene Polymorphisms With Obesity-Related Metabolic Complications Among Severely Obese Patients
P2860
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P2860
description
1972 nî lūn-bûn
@nan
1972 թուականի Յուլիսին հրատարակուած գիտական յօդուած
@hyw
1972 թվականի հուլիսին հրատարակված գիտական հոդված
@hy
1972年の論文
@ja
1972年論文
@yue
1972年論文
@zh-hant
1972年論文
@zh-hk
1972年論文
@zh-mo
1972年論文
@zh-tw
1972年论文
@wuu
name
Enzyme deficiency in cholesteryl ester storage idisease
@ast
Enzyme deficiency in cholesteryl ester storage idisease
@en
Enzyme deficiency in cholesteryl ester storage idisease
@nl
type
label
Enzyme deficiency in cholesteryl ester storage idisease
@ast
Enzyme deficiency in cholesteryl ester storage idisease
@en
Enzyme deficiency in cholesteryl ester storage idisease
@nl
prefLabel
Enzyme deficiency in cholesteryl ester storage idisease
@ast
Enzyme deficiency in cholesteryl ester storage idisease
@en
Enzyme deficiency in cholesteryl ester storage idisease
@nl
P2860
P356
P1476
Enzyme deficiency in cholesteryl ester storage idisease
@en
P2093
Fredrickson DS
P2860
P304
P356
10.1172/JCI106997
P407
P577
1972-07-01T00:00:00Z