title: Acid lipase and carboxylesterases in EBV-transformed lymphoid cell line from Wolman's disease: influence of fatty acid structure of substrate.
abstract: Lymphoid cell lines established by Epstein-Barr virus transformation of blood B lymphocytes from a patient with Wolman's disease exhibited the acid lipase deficiency characteristic for this disease. Comparison of hydrolysis by normal and Wolman's cells of 4-methylumbelliferyl-acyl esters with variable chain length demonstrates that: (1) the best substrates for acid lipase were characterized by an acyl chain length of 12-18 carbon atoms; (2) the acid residual activity in Wolman's cells showed a slightly different substrate specificity and this is probably due to an acid carboxylesterase different from the lysosomal acid lipase, and (3) the 'nonspecific' carboxylesterases (at pH 6.0 and 8.0) not inhibited by taurocholate showed a characteristic substrate specificity for short-chain fatty acids. In the used assay conditions (optimal for acid lipase), methylumbelliferyl-palmitate, -elaidate and -lignocerate are the most accurate synthetic substrates for the diagnostic of Wolman's disease.
mesh_terms: D002265:Carboxylic Ester Hydrolases / Q000378:metabolism*; D002460:Cell Line; D002472:Cell Transformation, Viral; D005227:Fatty Acids / Q000378:metabolism; D004854:Herpesvirus 4, Human; D006801:Humans; D006923:Hymecromone / Q000378:metabolism; D008049:Lipase / Q000378:metabolism*; D008052:Lipid Metabolism, Inborn Errors / Q000201:enzymology*; D008214:Lymphocytes / Q000201:enzymology* / Q000382:microbiology; D013379:Substrate Specificity
chemical_list: D005227:Fatty Acids; D006923:Hymecromone; D002265:Carboxylic Ester Hydrolases; D008049:Lipase