wolman disease/phospholipid

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Progressive macrophage accumulation in lysosomal acid lipase deficiency.

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Lysosomal acid lipase (LAL) deficiency (LAL-D) is a lysosomal lipid storage disorder in which the accumulation of cholesteryl esters and triglycerides predominantly in hepatocytes and cells of the macrophage-monocyte system is observed. The disturbance in the synthesis and trafficking of cholesterol

Neutral lipid storage with acid lipase deficiency: a new variant of Wolman's disease with features of the Senior syndrome.

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A girl presented with small stature, obesity, tapetoretinal degeneration, deafness, psychomotor regression, seizures, acanthosis nigricans, hepatomegaly, and chronic tubulointerstitial nephropathy. She died at age ten with renal insufficiency and uncontrolled seizures. Histochemistry showed lipid

Lysosomal acid lipase deficiency impairs regulation of ABCA1 gene and formation of high density lipoproteins in cholesteryl ester storage disease.

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ATP-binding cassette transporter A1 (ABCA1) mediates the rate-limiting step in high density lipoprotein (HDL) particle formation, and its expression is regulated primarily by oxysterol-dependent activation of liver X receptors. We previously reported that ABCA1 expression and HDL formation are

Lysosomal acid lipase deficiency in rats: lipid analyses and lipase activities in liver and spleen.

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We report the biological characterization of an animal model of a genetic lipid storage disease analogous to human Wolman's disease. Affected rats accumulated cholesteryl esters (13.3-fold), free cholesterol (2.8-fold), and triglycerides (5.4-fold) in the liver, as well as cholesteryl esters

[Acid lipases and acid cholesterol esterases: Wolman's disease and cholesteryl ester storage disease].

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In the first part of the review are reported the properties of mammalian acid lipases and cholesterol esterases. Lysosomal acid lipase differs from the other acid or neutral lipases by its subcellular localization and a large substrate specificity on natural lipids, triglycerides and cholesteryl

Cellular uptake and catabolism of high-density-lipoprotein triacylglycerols in human cultured fibroblasts: degradation block in neutral lipid storage disease.

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High-density lipoprotein (HDL)-[3H]triolein (i.e. [3H]triolein incorporated into reconstituted HDL) was taken up by cultured fibroblasts through an apparently saturable process, competitively inhibited by non-labelled HDL and independent of the LDL receptor. Using 125I-HDL and HDL-[3H]triolein,

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