Liver fatty acid-binding protein in two cases of human lipid storage.
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 less than 0.02), respectively, as compared to controls. A possible role of L-FABP in the pathogenesis of RS is discussed.
Vergani
L
L
Department of Neurology, University of Padova, Italy.
Fanin
M
M
Martinuzzi
A
A
Galassi
A
A
Appi
A
A
Carrozzo
R
R
Rosa
M
M
Angelini
C
C
D000293:Adolescent; D000818:Animals; D000906:Antibodies / Q000276:immunology; D002352:Carrier Proteins / Q000276:immunology / Q000378:metabolism*; D002645:Chickens; D004797:Enzyme-Linked Immunosorbent Assay; D000071438:Fatty Acid-Binding Protein 7; D050556:Fatty Acid-Binding Proteins; D005260:Female; D006801:Humans; D008052:Lipid Metabolism, Inborn Errors / Q000378:metabolism* / Q000473:pathology; D008099:Liver / Q000648:ultrastructure; D008107:Liver Diseases / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D009363:Neoplasm Proteins; D012202:Reye Syndrome / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D025521:Tumor Suppressor Proteins
D000906:Antibodies; D002352:Carrier Proteins; C493319:FABP1 protein, human; C107608:FABP7 protein, human; C493320:Fabp1 protein, mouse; D000071438:Fatty Acid-Binding Protein 7; D050556:Fatty Acid-Binding Proteins; D009363:Neoplasm Proteins; D025521:Tumor Suppressor Proteins