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Lipid and lysosomal enzymes in human fibroblasts cultured with perhexiline maleate
Authors:Samia Albouz  Jeanne-Marie Boutry  Gisèle Dubois  Raymond Bourdon  Jean-Jacques Hauw  Nicole Baumann
Affiliation:(1) Laboratoire de Biochimie-Toxicologie, Hôpital Fernand-Widal, F-75651 Paris, France;(2) Laboratoire de Neuropathologie Charles Foix, Hôpital de la Salpêtrière, F-75651 Paris, France;(3) Laboratoire de Neurochimie, INSERM U.134, CNRS ERA 421, Hôpital de la Salpêtrière, F-75651 Paris, France
Abstract:Summary To understand the mechanism of the lysosomal lipid storage induced by perhexiline maleate, we performed simultaneous lipid analysis and lysosomal enzymes determinations. Human libroblasts were cultured for 5 days in the presence of perhexiline maleate at a concentration of 2 mgrg/ml of culture medium. Lipid analysis showed that those non toxic levels determined the same changes as seen with higher concentrations of the drug (Hauw et al. 1980) i. e. increase of cholesterol and of all major phospholipids. Qualitative phsopholipid pattern was not markedly changed. Lysosomal enzymes activities were not modified with the exception of sphingomyelinase which was reduced to 12% of its normal level.
Keywords:Perhexiline Maleate  Sphingomyelinase  Human fibroblast cultures
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