Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020473 (hyperlipidemia)
 15,891 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We describe a new phenotype of hyperlipoproteinemia in two members of a family with a high degree of consanguinity. Both have a history of uncontrolled diabetes mellitus without ketoacidosis, and a family history of coronary artery disease at a relatively early age. A high degree of insulin resistance was found. The abnormal lipoprotein(s) has alpha-lipoprotein mobility on cellulose acetate electrophoresis and has a relative density of less than 1.006 as determined by ultracentrifugation of serum collected after a short fast. The fraction isolated by ultracentrifugation contains about half of the serum cholesterol and triglycerides and most of the phospholipids; the major protein component is albumin. Immunoelectrophoresis showed low concentrations of beta-lipoproteins in both sera, and two abnormal precipitin bands against monospecific antiserum to antilipoprotein A; a third member of the family showed only one abnormal precipitin band against the same antibody. We tentatively propose an abnormal gene(s) as the underlying mechanism. The insulin-resistant diabetes mellitus, probably inherited separately, may aggravate the hyperlipidemia.
 ...
PMID:Hyperlipoproteinemia with albumin-lipid complex: a novel type of hyperlipoproteinemia associated with insulin-resistant diabetes mellitus. 397 55

Primary biliary cirrhosis (PBC) is a chronic nonsuppurative, destructive cholangitis, whose etiology is unknown. Morbidity arises early from pruritus and later from hypercholesterolemia with xanthoma formation. Therapy is supportive and directed at the complications of cholestasis. Plasmapheresis has been reported to benefit patients with hyperlipidemia and PBC; thus a pilot study of plasmapheresis utilizing the Haemonetics Model 30 with replacement by albumin and saline was conducted. Five patients (four female and one male) with a mean age of 43 (range 29-58) and a mean duration of illness of 9.5 years (range 6-21) with marked jaundice, xanthomas, xanthelasma, hepatomegaly, fatigability, anorexia, and pruritus, as well as mild nausea were studied. Peripheral neuropathy was present in two patients. Two patients had splenomegaly. Two patients had an associated Sjogren syndrome. All patients had high serum bilirubin, alkaline phosphatase, and cholesterol levels and mild elevations in aspartate amino transferase and alanine amino transferase activities. Immune complexes measured in four patients were present. Antimitochondrial antibody titers were significant in all patients. Patients underwent a mean of 63 plasmapheresis procedures over a mean of 112 weeks removing a mean of 94.7 liters of plasma. No serious toxicity was seen. All patients showed a reduction in pruritus, xanthomas, xanthelasmas, and serum cholesterol values. The two patients who had evidence of Sjogren syndrome noted subjective improvement. All patients who had fatigue, anorexia and nausea also noted moderate improvement. There was no change in hepatomegaly or splenomegaly in patients demonstrating such organomegaly. Liver function did not change significantly. Overall, four patients had improvement in their condition and one patient achieved stability.(ABSTRACT TRUNCATED AT 250 WORDS)
 ...
PMID:The clinical effectiveness and safety of chronic plasmapheresis in patients with primary biliary cirrhosis. 403 Jul 9

Studies were made on tryptophan metabolism in Nagase analbuminemic rats (NAR), a mutant strain established from Sprague-Dawley rats that is characterized by lack of plasma albumin and hyperlipidemia. The total tryptophan concentration in NAR plasma is one fifth of normal, but the plasma free tryptophan concentration and liver and brain tryptophan levels are normal. Radioimmunoelectrophoresis showed that the tryptophan binding protein in NAR plasma is alpha 2-macroglobulin. The effect of oral tryptophan loading on plasma tryptophan and urinary excretion of tryptophan and its metabolites were examined. Differences between the values for NAR and normal rats were not significant except that the disappearance rate of tryptophan from NAR plasma was faster than in normal rats. The effects of several drugs that influence the concentration of non-esterified fatty acid (NEFA) or amino acids in the plasma of normal rats on NAR plasma tryptophan and NEFA concentrations and brain tryptophan metabolism were investigated. These drugs did not alter plasma NEFA and free tryptophan in NAR in the same direction as in normal rats. This difference may be caused by the difference of tryptophan binding proteins in the plasma of these two strains.
 ...
PMID:Tryptophan metabolism in analbuminemic rats. 619 53

Transmural cholesterol concentrations and transmural distribution of intravenously injected 131I-albumin were measured simultaneously in the aorta of both normal and cholesterol-fed pigs. The characteristic profile of 131I-distribution across the normal pig aorta in areas of high and low permeability (as identified by Evans Blue dye) is modified by cholesterol feeding. Additionally, the transmural concentration of cholesterol, which is similar in areas of high and low permeability of the normal pig aorta, is remarkably higher in the intima of high permeability areas of aortas of cholesterol-fed pigs and suggests that differential permeability in focal areas of the aorta is important only under conditions of hyperlipemia.
 ...
PMID:Modification in the transmural concentration of cholesterol and the transmural distribution of 131I-albumin in the aortas of pigs fed cholesterol: a preliminary report. 620 43

An albumin-deficient and fatty strain of rats (AFR) was established from crosses between albumin-deficient rats (NAR) and fatty Zucker rats. AFRs have double homozygous mutant genes (alb/alb, fa/fa). The AFRs are heavier than the fatty Zucker rats and they have enlarged livers and adrenal glands. In addition, AFRs show more severe hyperlipidemia than fatty Zucker rats. This strain of rats may serve as a model of human obesity and hyperlipidemia.
 ...
PMID:Establishment of an albumin-deficient and fatty strain of rats. 646 10

To evaluate the usefulness of nephelometry in predicting hyperlipidemia in neonates receiving intravenous fat (IVF), 23 infants in our neonatal intensive care nursery had simultaneous measurements of the serum IVF level (as determined by nephelometry), triglyceride, cholesterol, and free fatty acid/albumin molar ratio. There was a positive correlation between the serum IVF level and triglycerides, but the IVF level did not reliably predict elevated triglycerides, cholesterol, or free fatty acid-albumin molar ratio. Thus, neonates receiving IVF emulsions cannot be monitored by nephelometry alone. Adequate monitoring requires measurement of specific lipid fractions.
 ...
PMID:Limited value of nephelometry in monitoring the administration of intravenous fat in neonates. 668 58

We have evaluated an affinity-chromatographic procedure for determination of glycated albumin (GA) and glycated total serum protein (GSP). Recovery of these analytes was inversely related to free glucose concentration, thus necessitating removal of free glucose. For this we used molecular-exclusion chromatography on G-25 Sephadex, or dialysis, the latter procedure resulting in significantly (p less than 0.05) lower concentrations of GSP and GA. Total protein concentration and percent glycation are also inversely related, and so protein concentrations must be standardized before the assay. Within- and between-run CVs for both GSP and GA were less than 6.5 and 18%, respectively, the determination of GA being generally the more precise of the two. Labile glycated fractions, lipemia, icterus, hemolysis, and type of anticoagulant did not affect the results, but assay temperature did. Diabetic subjects showed substantially higher concentrations of GA and GSP than did normal subjects. Because of the life span of these analytes in circulation, their measurement may provide a short-term index of glycemic control.
 ...
PMID:Quantification of nonenzymically glycated albumin and total serum protein by affinity chromatography. 669 94

In the antilipoprotein type of autoimmune hyperlipidemia (AIH), the immunoglobulins (Ig) are bound to lipoproteins by their antibody site and circulate as immune Ig-Lp complexes. In the earlier studies, the specific antibody activities were demonstrated in vitro by specific but rather sophisticated methods which were not suitable for the screening of antilipoprotein AIH in large populations. In the Ig-Lp test described here, the immunoglobulins bound to the low density lipoproteins (Ig-Lp) are detected by floating the complexes at D 1.10 in the ultracentrifuge in a physiological saline sucrose density gradient; delipidating them by ether in the presence of 0.2 M urea, and assaying the protein by radial immunodiffusion and laser immunonephelometry with antisera specific for IgG, IgA, IgM, low density lipoproteins and albumin. Radial immunodioffusion and immunonephelometry gave similar results. This Ig-Lp test was positive in 5 myelomas associated with hyperlipidemia, which were previously classified as AIH with specific methods. And the test was specific for the Ig type of the monoclonal antibody involved in each case (3 IgA, 1 IgG and 1 IgM). It was negative in 6 normolipidemic myelomas and also in 40 sera from healthy blood donors and one normal serum taken 4 hours after a fat meal. Although the Ig-Lp test is not specific for antilipoprotein antibodies, the results of this study allow to use if for the screening for AIH.
 ...
PMID:Antilipoprotein autoimmune hyperlipidemia. The Ig-Lp test. 677 97

Lipids of HDL (high density lipoproteins) and their subfractions (HDL2 and HDL3), and LCAT activity (lecithin: cholesterol acyltransferase) were determined in hepatobiliary diseases without severe hyperbilirubinemia (less than 10 mg/dl). The decrease in major lipid constituents (cholesterol and phospholipids) of HDL was mainly attributable to the decrease in those of HDL3, except in some liver diseases of acute or severe stage (acute hepatitis in an acute stage and hepatoma) which were accompanied with a simultaneous moderate decrease in those of HDL2 and in fatty liver which showed a preferential decrease in those of HDL2. The LCAT activity also decreased in several diseases. Some of the hepatobiliary diseases, on the contrary, showed an increase in HDL-triglycerides (mostly in HDL3 and in some diseases also in HDL2) which might participate to some extent in secondary hyperlipidemia in the liver parenchymal diseases, although they were the minor lipid constituents of HDL. From results that HDL3- but not HDL2-cholesterol levels significantly correlated with serum total protein, albumin and choline esterase, it was suggested that the decrease in large constituents of HDL, particularly of HDL3, is caused by hepatocellular dysfunction which causes inhibition of protein and lipid syntheses in the liver in most of the hepatobiliary diseases except for fatty liver which has a preferential decrease in HDL2 lipids.
 ...
PMID:Changes in high density lipoproteins in patients with hepatobiliary diseases. Levels and lipid composition of HDL2 and HDL3 and LCAT reaction. 685 43

It has been postulated that hyperlipidemia in the nephrotic syndrome is due to overproduction of lipoproteins and that low colloid osmotic pressure (due to hypoalbuminemia) triggers this. Secretion of very low density lipoproteins (VLDL) by cultured rat hepatocytes has been shown to be inhibited by albumin, globulins, and dextrans, but the effect did not correlate with osmolarity. In the present studies we tested the hypothesis that viscosity rather than osmolarity might be the parameter determining the effectiveness of macromolecules in inhibiting VLDL synthesis and secretion by cultured rat hepatocytes. Synthesis and secretion of VLDL was measured in terms of incorporation of [3H] glycerol into medium triglycerides and also from changes in the mass of secreted VLDL triglycerides and apoproteins. The viscosity of the culture medium was increased by addition of dextran-500, gelatin or methylcellulose MX 880. Synthesis and secretion of VLDL was inhibited in direct proportion to increasing viscosity. At a viscosity of 2, which is about that of normal plasma, VLDL secretion was reduced by 20%. An inhibition of 60-70% in secretion and 30-40% in synthesis of VLDL lipid and protein components was observed at a relative viscosity of approximately 3.7. This viscosity was obtained by addition of any of the following: 3% dextran, 3% gelatin, 0.2% methylcellulose, or a combination of 0.1% methylcellulose plus 2% gelatin. Thus, similar viscosities resulted in similar degrees of inhibition despite differences of up to 16-fold in mass concentration and up to 20-fold in osmolarity.
 ...
PMID:Viscosity of culture medium as a regulator of synthesis and secretion of very low density lipoproteins by cultured hepatocytes. 706 16


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>