Soluble guanylyl cyclase and platelet function.
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Animals↗
Biomedical subjects
G Wolfram
Publications and source records attributed to G Wolfram.
Enrichment of selected serum fatty acids after a small oral dosage of (1-13C)- and (8-13C)triolein in human volunteers analysed by gas chromatography/combustion isotope ratio mass spectrometry.
In this study the fate of 100 mg orally administered (1-13C)- and (8-13C)triolein was traced in the serum lipids of four healthy human subjects. After an overnight fast the subjects consumed hourly meals of a liquid formula diet over 12 h. Ninety minutes after the first meal in the first study (1-13C)triolein was given and in the repeat study the same subject received (8-13C)triolein. Triacylglycerol (TG), phospholipid (PL) and cholesterol ester (CE) were isolated from serum sampled prior to and in intervals after isotope administration. Fatty acid composition of serum lipids was measured using gas chromatography/mass spectrometry. 13C enrichment in palmitic, stearic, oleic and linoleic acids of these fractions was determined by gas chromatography/isotope ratio mass spectrometry. With (8-13C)triolein a significantly higher enrichment (peak +17.1 +/- 14.3/1000 delta 13C) was found in the oleic acid of TG fraction than with (1-13C)triolein (peak -7.1 +/- 4.2/1000), which may be due to a faster elimination of (1-13C)oleic acid from serum TG. 13C enrichments in the other fatty acids of the TG fraction as well as of PL and CE fractions were in the range of natural 13C abundance (-25 to -32/1000).
Adult↗
[Nutrient intake in permanent night shift workers].
By means of a modified weight record technique, total dietary intake was obtained in 24 permanent night-shift workers during a period of 7 consecutive days; intake data were attached to eight defined meals. As a control, 25 shift workers of the same company were investigated during the morning shift. Mean daily energy intake was 2866 +/- 616 kcal (12.0 +/- 2.6 MJ) including 14% protein, 39.5% fat, 37.8% carbohydrates, and 8.7% alcohol. Statistically significant differences between groups were found for vitamin E and polyunsaturated fatty acids. In both groups intake data for vitamin A, D, zinc and dietary fiber did not meet 3/4 of the recommendations of the German Nutrition Society. In contrast to controls, nutrient intake of the permanent night-shift workers was slightly less during weekend days; mean meal frequency decreased from 5.4 (weekday) to 4.3. The distribution of total daily nutrient intake to different meals partly did not agree with existing recommendations; however, the deviation seems reasonable. Regarding the risks for accidents as well as the working capacity, the relatively high alcohol intake during working hours has to be criticized.
Adult↗
Identification of defective binding of low density lipoprotein by the U937 proliferation assay in German patients with familial defective apolipoprotein B-100.
Familial defective apolipoprotein B-100 (FDB) is a dominantly inherited disorder characterized by decreased binding of low density lipoprotein (LDL) to the LDL receptor due to a substitution of glutamine for arginine in residue 3500 of apolipoprotein B-100. We present the results of the U937 cell proliferation assay for the detection of familial defective apo B100 in 13 German FDB patients. Due to a defect in the pathway of cholesterol synthesis the human myelomonocytic tumour cell line U937 lacks the ability to synthesize cholesterol which makes proliferation of these cells dependent on the presence of exogenous LDL-cholesterol. U937 cells were incubated with LDL from 13 FDB-patients, 10 healthy normocholesterolaemic individuals (NC) and 26 patients with familial hypercholesterolaemia due to a defective LDL-receptor (FH). At LDL-cholesterol concentrations below 1 microgram ml-1 no proliferation occurred. In the presence of LDL from FDB patients at concentrations between 2.5 micrograms ml-1 and 15.0 micrograms ml-1, the proliferation was significantly reduced compared to LDL from FH-patients and normocholesterolaemic controls. At 5 micrograms ml-1 the reduction was 31-80% regardless of age, sex, apo E genotype, Lp(a)- and lipid levels. At concentrations above 25.0 micrograms ml-1 no further differences were observed. The present results indicate that the U937 proliferation assay is a reliable test for the detection of defective LDL-binding due to the 3500 mutation in FDB patients. It may be useful for the detection of defective binding of LDL due to other mutations in the apo B-100 gene.
Adult↗
Recommendations on nutrient intake in Germany.
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Eating↗
[Is the value of antioxidative vitamins established?].
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Antioxidants↗
[Comparative study of long-term parenteral nutrition with medium-chain and long-chain triglycerides in post-aggression metabolism].
OBJECTIVE: To determine the effect of a pure glucose and of different glucose/fat regimens as nonprotein energy source on substrate metabolism, nitrogen balance, lipoprotein pattern and liver enzymes. Long-chain and mixed long-/medium-chain triglyceride emulsions as 10 and 20% solutions were infused. DESIGN: Prospective randomized study. SETTING: General ward of a university hospital. PATIENTS: 29 patients in five groups after colorectal surgery. INTERVENTIONS: According to Harris-Benedict an amount of 150% of the calculated daily calorie intake was infused. Besides nitrogen balance and routine laboratory tests the lipoprotein pattern was examined. RESULTS: No difference was observed in protein balance, while a pathological rise of liver enzymes was mainly seen with glucose 20% and long-chain fat emulsions in a concentration of 10%. Physiological lipoprotein balance could only be achieved with a 20% solution of long-chain and medium-chain emulsions. CONCLUSIONS: The results demonstrate a fast metabolism of the MCT/LCT 20% solution with physiological lipoprotein pattern and no change in liver enzymes. High-dose glucose infusions and long-chain fat emulsions may cause a fatty degeneration of the liver, and 10% MCT/LCT emulsions may cause a rise of phospholipids and a generation of lipoprotein X.
Adult↗
[Symposiums of the 99th Congress of the German Society of Internal Medicine. Nutritional therapy].
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Germany↗
Identification of the serine-156 to leucine mutation in the low-density lipoprotein receptor in a German family with familial hypercholesterolemia.
Familial hypercholesterolemia is caused by various mutations in the gene encoding the low-density lipoprotein receptor. To date more than 100 mutations have been identified, including insertions and deletions as well as single base changes. In the German population haplotype analysis using four restriction fragment length polymorphisms has recently suggested that there exist at least six different genetic defects. Screening 100 FH patients of German origin for the serine 156 to leucine mutation, originally described in a Puerto Rican family living in the United States, resulted in the identification of the mutation in one family. However, by haplotype analysis the mutation was found on a different haplotype from that reported originally. Based on comparison of the haplotypes and their frequencies we suggest that this mutation has occurred independently at least twice.
Adult↗
The effect of the apolipoprotein E polymorphism on lipid levels in patients with familial defective apolipoprotein B-100.
Serum lipid concentrations of patients with familial defective apolipoprotein B-100 (FDB) show a high interindividual variability although the underlying defect is caused by a single point mutation. On the other hand, several genetic factors modulating serum cholesterol levels are known, such as DNA polymorphisms of the apolipoprotein B or the apolipoprotein E (apo E) gene. To assess the effect of the apo E polymorphism on serum cholesterol, lipid levels of FDB patients (n = 36) were compared with those of a normolipidemic control group (n = 272) according to their apo E genotype. For the FDB group mean values of low-density lipoprotein (LDL) cholesterol (mg/dl) were 225.7 +/- 53.7 for E3/2 genotype (n = 3), 234.2 +/- 48.3 for E3/3 genotype (n = 20), and 252.4 +/- 73.8 for E4/3 genotype (n = 13). Means of triglycerides (mg/dl) were 121.0 +/- 21.2, 114.8 +/- 60.7, and 110.0 +/- 62.8 for the respective apo E genotypes. The calculated average effect of the apo E alleles on LDL cholesterol levels was -6.0% for allele e2 and +3.7% for e4 relative to the whole FDB group. The effect on triglyceride levels was +7.5% for e2 and -3.6% for e4. The control group showed a similar variation in LDL cholesterol depending on the different apo E genotypes. About 6% of the total variation in LDL cholesterol can be accounted for by the apo E locus in normolipidemic and hypercholesterolemic individuals alike.
Adolescent↗
Effects of fish oil concentrate on lipoproteins and apolipoproteins in familial combined hyperlipidemia.
The effects of two moderate doses of long-chain n-3 fatty acids (3.0 and 4.5 g EPA+DHA per day for 4 weeks each) on serum lipids and lipoproteins of patients with familial combined hyperlipidemia (FCH) were studied in a double-blind, placebo-controlled clinical trial. In nine patients with FCH n-3 fatty acids led to a statistically significant, dose-dependent fall in very low density lipoprotein (VLDL) triglycerides (3 g/day: -42%, 4.5 g/day: -55%) VLDL cholesterol (3 g/day: -41%, 4.5 g/day: -47%), and VLDL apolipoprotein (apo) B-100 (3 g/day: -40%, 4.5 g/day: -56%). No overall change in low-density lipoprotein (LDL) cholesterol was found, as confirmed statistically. However, when analyzing the data of single patients LDL cholesterol and LDL apo B did not change in five patients but increased dose dependently (from pretreatment 4.80 +/- 0.93 mmol/l to 5.70 +/- 0.93 mmol/l LDL cholesterol after 4.5 g/day) in four. LDL and VLDL composition as indicated by cholesterol/apo B-100 and triglyceride/apo B-100 ratios did not change significantly. High-density lipoprotein (HDL) cholesterol was unchanged; the HDL cholesterol/apo A-I+apo A-II ratio increased by 19% (P < 0.05) during fish oil treatment. We conclude that in FCH moderate doses of long-chain n-3 fatty acids are highly effective in lowering pathological VLDL triglycerides, VLDL cholesterol, and VLDL apo B. LDL cholesterol must, however, be monitored during treatment as it may rise substantially in some although not in all patients with this disease.
Adult↗
Identification of the 408 valine to methionine mutation in the low density lipoprotein receptor in a German family with familial hypercholesterolemia.
Familial hypercholesterolemia (FH) is caused by different mutations in the gene encoding the low density lipoprotein receptor (LDLR). In Caucasian patients, at least three single point mutations have been identified causing FH. The asparagine206 to glutamine, and valine408 to methionine mutations were originally described in Afrikaners and recently identified in Dutch FH patients. The proline664 to leucine mutations was previously identified in an FH homozygote of Asian Indian origin and later identified in patients from London. Any of these mutations can be identified using direct amplification of genomic DNA by the polymerase chain reaction (PCR) and restriction enzyme digestion of PCR products. In this study, 100 unrelated German FH patients were screened for these three mutations. The valine408 to methionine mutation was identified in one individual and subsequently in the hypercholesterolemic child of the proband. Haplotype analysis with 7 restriction fragment length polymorphisms (RFLPs) revealed that the mutant allele carried the same haplotype as the previously described patients in South Africa and the Netherlands. Our finding supports the previous assumption of the European origin of the mutation.
Adolescent↗
Dietary habits and serum lipids of a group of German amateur body-builders.
Dietary intake, nutrient supplementation, and serum lipids were investigated in 13 German male amateur body-builders during a non-competitive period. Dietary information was collected with weighted food records during 14 consecutive days. Daily energy intake was 17.1 +/- 3 MJ including 22 +/- 5% protein, 26 +/- 6% fat, and 49 +/- 4% carbohydrates. "Breads and cereals" and "milk and dairy products" revealed to be the most important food groups. Protein supplements contributed 13% of total protein intake. With food alone the average supply of the vitamins A, D, E and B1 was < 3/4 of the recommended amounts (DGE). Due to the high consumption of supplement preparations, total daily intake of most of the selected minerals and vitamins--particularly of vitamins of the B-group--increased far above recommended dietary intake. Mean fasting serum triglyceride, phospholipid and total cholesterol concentrations were in a normal range, while high-density lipoprotein cholesterol levels were reduced. The serum cholesterol ester fatty acids analysis confirmed a rather low intake of essential fatty acids (linoleic acid) found by dietary assessment. In conclusion, with a few corrections in food selection patterns of the body-builders, a well balanced diet would be achieved and the use of nutrient supplementation products would become totally superfluous.
Adult↗
Relation of lipoprotein(a) to coronary heart disease and duplexsonographic findings of the carotid arteries in heterozygous familial hypercholesterolemia.
In familial hypercholesterolemia (FH) elevated Lp(a) concentrations are more frequent than in the general Caucasian population, but the clinical relevance of Lp(a) as a risk-factor in this group of patients is controversial. In 91 adult patients with heterozygous FH due to LDL-receptor defect we analyzed the correlation between Lp(a) concentrations, presence of coronary heart disease (CHD) and degree of atherosclerosis of the carotid arteries assessed by duplex scan. Coronary heart disease was present in 32 patients (24 males, 8 females). In the group without CHD the median of the Lp(a) distribution was 23 mg/dl, in the group with CHD 43 mg/dl (P < 0.05). The median of Lp(a) was 8 mg/dl in patients without pathological changes in the duplex scan of the carotids, 13 mg/dl in the group with intimal thickening, 25 mg/dl in patients with non-obstructing plaques, and 45 mg/dl in presence of > 30% luminal obstruction (P < 0.01). The role of Lp(a) as an independent risk factor was analyzed by stepwise logistic regression together with age, sex, LDL-, HDL-cholesterol, serum triglycerides, smoking status and presence of hypertension. For the prediction of CHD only age, HDL cholesterol and gender reached statistical significance. Lp(a) was, however, the lipoprotein parameter with the highest discriminative strength for the presence of a pathological duplex scan (P = 0.016), followed by LDL- (P = 0.03), and HDL-cholesterol (P = 0.03). These results provide direct evidence for a close correlation between Lp(a) and the rate of progression of atherosclerosis in FH, already at early, asymtomatic stages.
Adult↗
Different (13)CO(2) recovery of orally administered [1-(13)C]- and [8-(13)C] triolein in postprandial humans: an effect of phosphoenolpyruvate-carboxykinase (EC 4.1.1.32) in peripheral tissues?
In this study the conversion of orally administered [1-(13)C]- and [8-(13)C]triolein to CO(2) was compared in normal postprandial human subjects (3 female, 3 male). After an overnight fast the subjects consumed hourly meals of a liquid formula diet over 12 h (8.3% of the predicted 24 h resting energy expenditure/h). 90 min after the first meal on one occasion a bolus of [1-(13)C]triolein was given and in the repeat study the same subject received [8-(13)C]triolein. The order of isotope substrate was randomized. Isotope ratio mass spectrometry analysis of breath samples an recorded CO(2) production rate resulted in a significant 1.31 times greater (13)CO(2) recovery of [1-(13)C]triolein compared to [8-(13)C]triolein within 7.5 h. 10 h after bolus the significant difference disappeared. The different (13)CO(2) recovery is probably due to a different metabolic fate of (13)C at odd and even numbered carbon positions in the fatty acid chain caused by beta-oxidation, citric acid cycle and the phosphoenolpyruvate carboxykinase (EC 4.1.1.32) reaction in peripheral tissues. A contribution of a chain shortening in peroxisomes seems unlikely.
Journal Article↗
Odd-numbered medium-chain triglycerides (trinonanoin) in total parenteral nutrition: parameters of carbohydrate and protein metabolism.
To investigate the metabolic effects of intravenously administered odd-numbered medium-chain triglycerides in healthy non-stressed animals, trinonanoin/long-chain triglyceride (LCT) (7/3) fat emulsions were given in a high dose (46.5% energy) to rabbits (n = 8) for 11 days within a total parenteral nutrition regimen. In comparison with medium-chain triglyceride (MCT)/LCT fat emulsions, higher plasma glucose (day 4), plasma lactate and pyruvate (day 11) and liver glycogen concentrations were found for the test group, indicating a glucogenic effect of the trinonanoin/LCT emulsion. With the present study design, nitrogen balance of the rabbits was not significantly influenced by the kind of fat emulsion administered.
Animals↗
Organ changes after intravenous trinonanoin administration in rabbits.
For 11 days, an odd-numbered medium-chain triglyceride (trinonanoin, C9TG)/long-chain triglyceride (LCT) emulsion was given parenterally to rabbits, providing 46.5% of total energy. In comparison with LCT or MCT/LCT fat emulsions, no negative effects on organ weights and liver lipid concentrations were noted. In some rabbits of the C9TG/LCT group macroscopically but not histologically visible liver changes were observed. The extent of nonanoic acid deposition in liver triglycerides, liver phospholipids, adipose tissue and skeletal muscle after C9TG/LCT administration was similar to C8/C10 deposition in the MCT/LCT group; the enrichment of adipose tissue with 16 mol% was higher than expected.
Adipose Tissue↗
Independent mutation of arginine(3500)-->glutamine associated with familial defective apolipoprotein B-100.
Familial defective apolipoprotein B-100 (FDB) is characterized by a decreased affinity of low density lipoprotein (LDL) to the LDL receptor resulting in a dominantly inherited increase of plasma LDL. It is postulated that FDB is caused by a G to A mutation at nucleotide 10,708 in exon 26 of the apoB gene creating a substitution of glutamine for arginine in amino acid 3500. The arginine(3500)-->glutamine mutation has been identified on the same haplotype of the apoB gene in several populations from North America and Europe, suggesting that it occurred on a single ancestral gene. Independent mutations were not observed. The purpose of this paper is to report on a family where individuals show a dominantly inherited increase of plasma LDL associated with an independent arginine(3500)-->glutamine mutation as determined by haplotype analysis using polymorphic markers of the apoB gene. The identification of these individuals is strong evidence that the arginine(3500)-->glutamine mutation is causative for the defective binding of apoB-100.
Adult↗