Liver fatty acid-binding protein in two cases of human lipid storage |
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Authors: | Lodovica Vergani Marina Fanin Andrea Martinuzzi Andrea Galassi Andrea Appi Rosalba Carrozzo Maurizio Rosa Corrado Angelini |
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Institution: | (1) Regional Center for Neuromuscular Diseases, Department of Neurology, University of Padova, Italy;(2) Morbid Pathology, Hospital of Bassano, Italy;(3) Dept. of Neurology, University of Padova, Via Giustiniani 5, 35100 Padova, Italy |
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Abstract: | Summary FABPs in the various tissues play an important role in the intracellular fatty acid transport and metabolism. Reye's syndrome (RS) and multisystemic lipid storage (MLS) are human disorders characterized by a disturbance of lipid metabolism of unknown etiology. We investigated for the first time L-FABP in these two conditions. Affinity purified antibodies against chicken L-FABP were raised in rabbits, and found to cross-react specifically with partially purified human L-FABP. L-FABP content in liver samples of two patients with RS and MLS was investigated by immuno-histochemistry, SDS-PAGE and ELISA. L-FABP immuno-histochemistry showed increased reactivity in the liver of RS patient and normal reactivity in MLS liver. L-FABP increase in RS liver was confirmed by densitometry of SDS-PAGE and ELISA method. By these two methods the increase amounted to 180% and 199% (p < 0.02), respectively, as compared to controls. A possible role of L-FABP in the pathogenesis of RS is discussed. |
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Keywords: | FABP L-FABP Reye's syndrome multisystemic lipid storage fatty liver |
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