title: The effect of three serum basic proteins on the mass of lipids in normal and hyperapoB fibroblasts.
abstract: We studied whether serum basic proteins (BPs) produce abnormal changes in the mass of cellular lipids in fibroblasts from patients with hyperapobetalipoproteinemia (hyperapoB) and if inhibition or stimulation of protein kinase C affects these processes. In normal cells, BP I increased the mean mass of triglycerides about twofold, whereas there was significantly less stimulation in hyperapoB cells (P < .005). The increase in the mass of cell cholesteryl esters seen in normal cells with BP I was also significantly reduced in hyperapoB cells (P < .005). In contrast, BP II abnormally stimulated the mass of cell cholesteryl esters sixfold in hyperapoB cells (P < .005). BP I also stimulated the mass of total phospholipids about twofold in normal cells, an effect that was reduced by about one third in hyperapoB cells (P = .08). No abnormality was found in hyperapoB cells with BP III. H-7, an inhibitor of protein kinase C, decreased the effects of BP I and BP II in normal and hyperapoB cells. C:8, an analogue of diacylglycerol, activated protein kinase C and stimulated triglyceride formation in normal (fourfold) and hyperapoB (fivefold) cells in the absence of BP I. When added with C:8, BP I further increased triglyceride production 1.5-fold in normal cells but not in hyperapoB cells. Two cellular abnormalities in lipid metabolism in hyperapoB fibroblasts were found, one with BP I, another with BP II. Protein kinase C activity was not deficient in hyperapoB cells, and the defect(s) may occur at another, perhaps earlier, step in the pathway.
authors.affiliation: Department of Pediatrics, Johns Hopkins University Hospital, Baltimore, MD 21287-3654.
authors.forename: P O
authors.initials: PO
authors.lastname: Kwiterovich
authors.forename: M
authors.initials: M
authors.lastname: Motevalli
authors.forename: M
authors.initials: M
authors.lastname: Miller
mesh_terms: D019307:1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine; D000328:Adult; D001798:Blood Proteins / Q000494:pharmacology*; D002784:Cholesterol / Q000378:metabolism; D002788:Cholesterol Esters / Q000378:metabolism; D004789:Enzyme Activation; D005260:Female; D005347:Fibroblasts / Q000187:drug effects / Q000378:metabolism*; D006801:Humans; D006938:Hyperlipoproteinemia Type II / Q000378:metabolism*; D007546:Isoquinolines / Q000494:pharmacology; D050356:Lipid Metabolism; D008297:Male; D009829:Oleic Acids / Q000378:metabolism; D010743:Phospholipids / Q000378:metabolism; D010879:Piperazines / Q000494:pharmacology; D011493:Protein Kinase C / Q000037:antagonists & inhibitors / Q000378:metabolism; D014280:Triglycerides / Q000378:metabolism
chemical_list: D001798:Blood Proteins; D002788:Cholesterol Esters; D007546:Isoquinolines; D009829:Oleic Acids; D010743:Phospholipids; D010879:Piperazines; D014280:Triglycerides; D019307:1-(5-Isoquinolinesulfonyl)-2-Methylpiperazine; D002784:Cholesterol; D011493:Protein Kinase C