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

Journal Article · Sat Sep 01 04:00:00 EDT 1990 · Journal of Lipid Research; (USA)

OSTI ID:6275934

Kuriyama, M; Yoshida, H; Suzuki, M; Fujiyama, J; Igata, A ^[1]

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1. Kagoshima Univ. School of Medicine (Japan)

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 (2.5-fold) and free cholesterol (1.33-fold) in the spleen. Triglycerides did not accumulate, and the levels actually decreased in the spleen. Analysis of the fatty acid composition of the cholesteryl esters and triglycerides showed high percentages of linoleic acid (18:2) and arachidonic acid (20:4) in both organs, especially in the liver. No accumulation of phospholipids, neutral glycosphingolipids, or gangliosides was found in the affected rats. Acid lipase activity for (14C)triolein, (14C)cholesteryl oleate, and 4-methyl-umbelliferyl oleate was deficient in both the liver and spleen of affected rats. Lipase activity at neutral pH was normal in both liver and spleen. Heterozygous rats showed intermediate utilization of these substrates in both organs at levels between those for affected rats and those for normal controls, although they did not accumulate any lipids. These data suggest that these rats represent an animal counterpart of Wolman's disease in humans.

• OSTI ID: 6275934
• Journal Information: Journal of Lipid Research; (USA), Journal Name: Journal of Lipid Research; (USA) Vol. 31:9; ISSN JLPRA; ISSN 0022-2275
• Country of Publication: United States
• Language: English

Related Subjects

550201* -- Biochemistry-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
ARACHIDONIC ACID
BIOLOGICAL ACCUMULATION
BIOLOGICAL MODELS
BODY
CARBON 14 COMPOUNDS
CARBOXYLESTERASES
CARBOXYLIC ACIDS
CHOLESTEROL
DIGESTIVE SYSTEM
DISEASES
ENZYME ACTIVITY
ENZYMES
ESTERASES
ESTERS
GLANDS
HYDROLASES
HYDROXY COMPOUNDS
ISOTOPE APPLICATIONS
LABELLED COMPOUNDS
LINOLEIC ACID
LIPASES
LIPIDS
LIVER
METABOLIC DISEASES
MONOCARBOXYLIC ACIDS
OILS
OLEIC ACID
ORGANIC ACIDS
ORGANIC COMPOUNDS
ORGANS
OTHER ORGANIC COMPOUNDS
PATHOGENESIS
SPLEEN
STEROIDS
STEROLS
TRACER TECHNIQUES
TRIGLYCERIDES
TRIOLEIN