Lymphoid cell lines as a model system for the study of Wolman's disease: Enzymatic, metabolic and ultrastructural investigations
- 5 November 1985
- journal article
- research article
- Published by Wiley in Journal of Inherited Metabolic Disease
- Vol. 9 (2), 193-201
- https://doi.org/10.1007/bf01799458
Abstract
Epstein-Barr virus (EBV) transformed lymphoid cell lines (LCL) were established from blood lymphocytes of a patient affected with Wolman's disease (WD) and from her parents. These LCL showed a severe deficiency in acid lipase activity using every substrate in comparison to LCL from normal subjects, in which acid lipase activity was similar to that in blood lymphocytes. In the LCL from Wolman's disease a major accumulation of neutral lipids was observed, mainly cholesteryl esters, CE (amount around 7 times higher than in normal cells and ratio of esterified/free cholesterol increased by 10 times) and to a lesser extent triglycerides, TG (amount increased by 1.5 times). Electron microscopy showed the storage vacuoles of neutral lipids quite characteristic of this lysosomal disease. The reported data demonstrated the validity of transformed LCL as a cellular model system in culture for experimental studies of Wolman's disease and for investigating the lysosomal metabolism of neutral lipids.This publication has 25 references indexed in Scilit:
- Enzyme studies on Epstein-Barr virus-transformed lymphoid cell lines from Wolman's diseaseBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1984
- Sphingomyelinase and nonspecific phosphodiesterase activities in epsteinbarr virus-transformed lymphoid cell lines from niemann-pick disease A, B and CBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1984
- Differentiation of microsomal from lysosomal triacylglycerol lipase activities in rat liverBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1983
- Lymphoblastoid cell lines, transformed by Epstein-Barr virus, in the enzymatic study of hereditary lysosomal storage diseases.The Tohoku Journal of Experimental Medicine, 1981
- Acid lipase activity of human lymphocytesBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1979
- Synthesis of trinitrophenylaminolauric acid and the use of its glyceryl esters for assaying lipase by a spectrophotometric procedureBiochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism, 1978
- A Rapid and Sensitive Method for the Quantitation of Microgram Quantities of Protein Utilizing the Principle of Protein-Dye BindingAnalytical Biochemistry, 1976
- A rapid and sensitive method for the quantitation of microgram quantities of protein utilizing the principle of protein-dye bindingAnalytical Biochemistry, 1976
- Dosage en deux temps du cholesterol total et du cholesterol libre plasmatiques par chromatographie gaz-liquideClinica Chimica Acta; International Journal of Clinical Chemistry, 1976
- Deficiency of an Acid Lipase in Wolman's DiseaseNature, 1969