Abstract:
:We have studied the recognition and uptake of acid lipase by human fibroblasts in order to determine requirements for localization and function of the enzyme in lysosomes. Our approach was based on evidence that a number of acid hydrolases involved in mucopolysaccharide metabolism are secreted from cultured fibroblasts and endocytosed by a phosphomannosyl-dependent, receptor-mediated process. Acid fatty acid ester hydrolase activity secreted from human fibroblasts was separable into two major forms by hydrophobic chromatography. The dominant form from normal cells was deficient in fibroblasts from patients with Wolman's disease, an inherited disorder of lysosomal cholesteryl ester and triglyceride metabolism. The time- and temperature-dependent, saturable uptake of this enzyme by fibroblasts was competitively inhibited by mannose 6-phosphate and was destroyed by pretreatment of the enzyme with phosphatase. Internalized lipase had a half-life of 1 day. Application of the enzyme to Wolman's disease fibroblasts reduced cholesteryl ester storage; this effect was blocked by ammonium chloride, a general inhibitor of lysosomal hydrolysis. Our results indicate that extracellular acid lipase is transported to fibroblast lysosomes by the same receptor-mediated process that functions in the packaging of several lysosomal glycosidases.
journal_name
J Lipid Resjournal_title
Journal of lipid researchauthors
Sando GN,Henke VLsubject
Has Abstractpub_date
1982-01-01 00:00:00pages
114-23issue
1eissn
0022-2275issn
1539-7262journal_volume
23pub_type
杂志文章abstract::Quantitatively and rapidly analyzing lipids is necessary to elucidate their biological functions. Herein, we developed a quantitative method for various lipid classes using supercritical fluid chromatography (SFC) coupled with a charged aerosol detector (CAD), providing high-throughput data analysis to detect a large ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.D094516
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journal_title:Journal of lipid research
pub_type: 杂志文章
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pub_type: 临床试验,杂志文章
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abstract::Murine strains susceptible to atherosclerosis provide valuable models to study factors involved in atherogenesis. In some murine models, limited hypercholesterolemia can be achieved and lesions develop primarily in the aortic origin, in the vicinity of the aortic valve. In other models such as LDL receptor-deficient a...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1995-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M084947
更新日期:2018-09-01 00:00:00
abstract::The in vivo formation of the sulfate ester of glycolithocholate is a critical step in the elimination of this hepatotoxic bile salt. Rhesus monkeys fed chenodeoxycholate or ursodeoxycholate, the precursors of lithocholate, develop frank cirrhosis in association with accumulation of nonsulfated glycolithocholate in bil...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1986-11-01 00:00:00
abstract::In studies of cebus monkey plasma lipoproteins, we have used an ultracentrifugally generated density gradient to isolate two distinct species of low density lipoproteins (LDL). Compositional analyses revealed that each of the ultracentrifugally isolated fractions was enriched in cholesteryl esters and contained a sing...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1989-05-01 00:00:00
abstract::Lysosomal acid lipase (LAL) is essential for the hydrolysis of triglycerides (TG) and cholesteryl esters (CE) in lysosomes. A mouse model created by gene targeting produces no LAL mRNA, protein, or enzyme activity. The lal-/- mice appear normal at birth, survive into adulthood, and are fertile. Massive storage of TG a...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:2001-04-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1972-01-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1997-01-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.S092007
更新日期:2019-04-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1993-09-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.D119000318
更新日期:2020-01-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M074997
更新日期:2017-05-01 00:00:00
abstract::Reduced white adipose tissue (WAT) LPL activity delays plasma clearance of TG-rich lipoproteins (TRLs). We reported the secretion of apoC-I, an LPL inhibitor, from WAT ex vivo in women. Therefore we hypothesized that WAT-secreted apoC-I associates with reduced WAT LPL activity and TRL clearance. WAT apoC-I secretion a...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.P064170
更新日期:2016-06-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1997-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1992-07-01 00:00:00
abstract::Mature endothelial lipase (EL) is a 68 kDa glycoprotein. In HepG2 cells infected with adenovirus encoding human EL, the mature EL was detectable in the cell lysates and heparin-releasable fractions. In contrast, cell media of these cells contained two EL fragments: an N-terminal 40 kDa fragment and a C-terminal 28 kDa...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400500-JLR200
更新日期:2005-05-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M600364-JLR200
更新日期:2007-07-01 00:00:00
abstract::Lipid transfer inhibitor protein (LTIP) is a regulator of cholesteryl ester transfer protein (CETP) function. Factors affecting plasma LTIP levels are poorly understood. In humans, plasma LTIP is elevated in hypercholesterolemia. To define possible mechanisms by which hyperlipidemia modifies LTIP, we investigated the ...
journal_title:Journal of lipid research
pub_type: 杂志文章
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journal_title:Journal of lipid research
pub_type: 杂志文章
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更新日期:2018-05-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:10.1194/jlr.M400423-JLR200
更新日期:2005-05-01 00:00:00
abstract::The chemical and biochemical properties of cholesterol-enriched and cholesterol-poor chylomicrons from rat lymph have been compared. The enriched particles, prepared from cholesterol-containing lipid dispersions, passed into the duodenum, had four to ten times the cholesteryl ester content of the control chylomicrons ...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1978-08-01 00:00:00
abstract::Insulin-dependent diabetes mellitus (IDDM) is associated with elevated plasma triglyceride levels that normalize after insulin administration. The observation that overexpression of the apoC-III gene in transgenic mice can cause hypertriglyceridemia and other evidence implicating apoC-III in the regulation of triglyce...
journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1994-11-01 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章
doi:
更新日期:1974-11-01 00:00:00
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pub_type: 杂志文章
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更新日期:2011-03-01 00:00:00
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pub_type: 杂志文章
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更新日期:2020-11-21 00:00:00
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journal_title:Journal of lipid research
pub_type: 杂志文章,评审
doi:10.1194/jlr.R004739
更新日期:2010-08-01 00:00:00