Establishment of genetic lipid storage disease with lysosomal acid lipase deficiency in rats

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We established a new animal model of a genetic lipid storage disease analogous to human Wolman's disease. Affected Donryu rats, who inherited the disease in an autosomal recessive mode, manifested marked hepatosplenomegaly, lymph node enlargement, and thickened, dilated intestine. Morphologically, many characteristic foam cells Were observed in livers and spleens. No adrenal calcification could be found in affected rats. Biochemical studies on spleen and liver tissues showed massive accumulation of esterified cholesterol and triglycerides and deficiency of acid lipase for [^<14>C] -triolen, [^<14>C]-cholesterol oleate, and 4-methylumbelliferyl oleate.This animal model could contribute greatly to the clarification of the physiological and pathological roles of lysosomal acid lipase in the metabolism of lipoproteins and cholesterol, and of the pathogeneisis of atherosclerosis.