Acid lipase cross-reacting material in Wolman disease and cholesterol ester storage disease.
Copyright policy )Acid lipase cross-reacting material in Wolman disease and cholesterol ester storage disease.
Material cross-reacting with antibodies to acid lipase was demonstrated in fibroblasts of three patients with Wolman disease and three with cholesterol ester storage disease. Quantitation of the immunologically cross-reacting material (CRM) by a single radial immunodiffusion method revealed normal levels in both mutant cell types. CRM specific activity toward triolein and cholesteryl oleate was reduced about 200-fold in the Wolman disease fibroblasts and 50- to 100-fold in the cholesterol ester storage disease cells when compared to normal.
Immunodiffusion, Lipase, Cross Reactions, Fibroblasts, Lipid Metabolism, Inborn Errors, Substrate Specificity, Humans, Cholesterol Esters, Lysosomes, Immunoelectrophoresis, Cells, Cultured, Triolein, Skin
Immunodiffusion, Lipase, Cross Reactions, Fibroblasts, Lipid Metabolism, Inborn Errors, Substrate Specificity, Humans, Cholesterol Esters, Lysosomes, Immunoelectrophoresis, Cells, Cultured, Triolein, Skin
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