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61.
Familial Mediterranean Fever (FMF) is an autosomal recessive genetic disorder characterised by recurrent and self-limited abdominal pain, synovitis and pleuritis. MEFV gene mutations are responsible from the disease and its protein product, pyrin or marenostrin, plays an essential role in the regulation of the inflammatory reactions. MEFV gene contains 10 exons and most of the mutations have been found on the last exon. Up to date, 152 mutations and polymorpisms have been reported inwhere V726A, M694V, M694I, M680I and E148Q are the most common mutations. In this study, MEFV allele frequencies of 136 individuals (60 from Pediatry, 76 from Internal Medicine) have been evaluated, and compared with each other. Asymptomatic individuals with FMF family history (4 from Pediatry, 6 from Internal Medicine) were excluded from the analysis. The prominent mutations indicated in the Pediatry group are V726A, M694V and M680I (G/C) and with the allele frequency of 0.06, 0.05 and 0.04 respectively while they were E148Q, M694V, M680I (G/C) in the Internal Medicine group with the allele frequency of 0.12, 0.08 and 0.04. The E148Q mutation is significantly overrepresented in the adult referrals (= 0.02). Mutation on both alleles was observed in only 12% of cases. Overall mutation frequency was low, seen in 26.2% (66/252). However, when only diagnosed patients were analyzed it is 72.7% (16/22). It is also interesting that 63% of individuals are female that there may be sex influence on FMF phenotype.  相似文献   
62.
Sulfites, which are commonly used as preservatives, are continuously formed in the body during the metabolism of sulfur-containing amino acids. Sulfite oxidase (SOX) is an essential enzyme in the pathway of the oxidative degradation of sulfite to sulfate protecting cells from sulfite toxicity. This article investigated the effect of sulfite on total antioxidant capacity (TAC), total oxidant status, lipid hydroperoxide (LOOH), and total free sulfydryl groups (-SH) levels in normal and SOX-deficient male albino rat plasma. For this purpose, rats were divided into four groups: control, sulfite-treated, SOX-deficient, and sulfite-treated SOX-deficient groups. SOX deficiency was established by feeding rats a low molybdenum diet and adding to their drinking water 200 ppm tungsten. Sulfite (70 mg/kg) was administered to the animals via their drinking water. SOX deficiency together with sulfite treatment caused a significant increase in the plasma LOOH and total oxidant status levels. -SH content of rat plasma significantly decreased by both sulfite treatment and SOX deficiency compared to the control. There was also a significant decrease in plasma TAC level by sulfite treatment. In conclusion, sulfite treatment affects the antioxidant/oxidant balance of the plasma cells of the rats toward oxidants in SOX-deficient groups.  相似文献   
63.
Liver X receptors (LXRs) are important regulators of cholesterol and lipid metabolism. LXR agonists have been shown to limit the cellular cholesterol content by inducing reverse cholesterol transport, increasing bile acid production, and inhibiting intestinal cholesterol absorption. Most of them, however, also increase lipogenesis via sterol regulatory element-binding protein-1c (SREBP1c) and carbohydrate response element-binding protein activation resulting in hypertriglyceridemia and liver steatosis. We report on the antiatherogenic properties of the steroidal liver X receptor agonist N,N-dimethyl-3beta-hydroxy-cholenamide (DMHCA) in apolipoprotein E (apoE)-deficient mice. Long-term administration of DMHCA (11 weeks) significantly reduced lesion formation in male and female apoE-null mice. Notably, DMHCA neither increased hepatic triglyceride (TG) levels in male nor female apoE-deficient mice. ATP binding cassette transporter A1 and G1 and cholesterol 7alpha-hydroxylase mRNA abundances were increased, whereas SREBP1c mRNA expression was unchanged in liver, and even decreased in macrophages and intestine. Short-term treatment revealed even higher changes on mRNA regulation. Our data provide evidence that DMHCA is a strong candidate as therapeutic agent for the treatment or prevention of atherosclerosis, circumventing the negative side effects of other LXR agonists.  相似文献   
64.
The aim of this study was to evaluate effects of dietary zinc and l-arginine supplementation on blood total antioxidant capacity (TAC), malondialdehyde (MDA), nitric oxide (NO), some blood chemistry parameters, and egg weights of laying quails. Three groups of Japanese quails were fed with a diet containing l-arginine (5 mg/kg), zinc (60 mg/kg), and normal basal diet (control) for 30 days. TAC, lipid peroxidation, and biochemical analysis were performed in the blood of animals. l-Arginine and zinc supplementation improved TAC and reduced MDA concentrations compared to the control (P?<?0.05). In comparison to the control, blood NO concentrations were increased by l-arginine (P?<?0.01) and zinc treatment (P?<?0.05). Both zinc (P?<?0.001) and l-arginine (P?<?0.01) supplementation significantly increased egg weight in laying quails. Some of the blood chemistry parameters were also altered by the treatment of l-arginine and zinc supplementation. No difference was found in blood albumin and creatinine levels among the groups. Blood glucose (P?=?0.833) and total protein (P?=?0.264) levels in control and l-arginine-treated groups were found to be similar. Glucose and total protein levels were decreased in zinc-supplemented animals compared to the control and l-arginine groups (P?<?0.05). No difference was found in triglyceride levels between control and zinc-applied groups (P?=?0.197). However, l-arginine treatment reduced the blood triglyceride levels compared to the control (P?<?0.05). In conclusion, l-arginine and zinc supplementation could be beneficial and effective for decreasing oxidative stress, boosting antioxidant capacity, and improving egg weight in the blood of the animals.  相似文献   
65.
Laccase enzyme (L) from Trametes versicolor was entrapped in three hydrogel structures namely poly(acrylamide-N-isopropylacrylamide), P(AAm-NIPA), and semi-interpenetrating networks of poly(acrylamide)/alginate, P(AAm)/Alg, and poly(acrylamide-N-isopropylacrylamide)/alginate, P(AAm-NIPA)/Alg. The optimum temperatures for free and all immobilized systems were found to be 40 °C. For free and immobilized laccase systems of P(AAm-NIPA)-L, P(AAm)/Alg-L and P(AAm-NIPA)/Alg-L, Km values were found to be 6.7 × 10?3, 8.8 × 10?2, 5.5 × 10?2 and 1.8 × 10?2 mM; Vmax values were calculated as 1.8 × 10?3, 2.5 × 10?2, 1.5 × 10?2 and 6.1 × 10?3 mM min?1, respectively. For free and the same immobilized systems, the enzymes retained 42%, 91%, 79% and 86% of their initial activities at the end of 56 days of storage. After using the mentioned immobilized systems repeatedly 10 times, they retained 77%, 71% and 84% of their original activities, respectively. For free and the same immobilized systems, decolorization of Acid Orange 52 (AO52) in 6 h were found to be 63%, 50%, 48% and 66%, respectively. Addition of 2,2′-azino-bis-(3-ethylbenzothiazoline-6-sulfonic acid), ABTS, into the assay medium increased these values up to 73%, 73%, 74% and 75%, respectively.  相似文献   
66.
Dyslipidemia in patients with glycogen storage disease types Ia (GSD Ia) and III (GSD III) does not lead to premature atherosclerosis. The aim of this study was to investigate the association among serum copper (Cu), zinc (Zn), iron (Fe), and selenium (Se) concentrations, and their carrier proteins: ceruloplasmin, albumin, and related antioxidant enzyme activities [superoxide dismutase (SOD), catalase (CAT), glutathione peroxidase (GPx), paraoxonase (PON), and arylesterase (ARYL)] in 20 GSD Ia and 14 III patients compared to age and sex matched 20 healthy subjects. Erythrocyte oxidative stress was measured by erythrocyte thiobarbituric acid reactive substances (eTBARSs). Hypertriglyceridemia [333 (36–890) mg/dL] in GSD Ia and hypercholesterolemia with elevated LDL-cholesterol [188 (91–313) mg/dL] and decreased HDL-cholesterol [32(23–58) mg/dL] levels in GSD III were found. Serum Cu, Fe, and Zn showed no significant differences between groups. However, Se 60 (54–94), 81 (57–127) μg/L, ceruloplasmin 21 (10–90), 27 (23–65) μg/L, and albumin 2.4 (1.7–5.1), 2.8 (1.8–4.06) g/dL levels were decreased in GSD Ia and III groups, respectively, in comparison with the controls [Se 110 (60–136) μg/L, ceruloplasmin 72 (32–94) μg/L, and albumin 4.4 (4–4.8) g/dL)]. In spite of high oxidative stress in erythrocyte detected by elevated eTBARS/Hb levels in GSD group [674.8 (454.6–948.2) for GSD Ia, 636.3 (460.9–842.1) for GSD III, and 525.6 (449.2–612.6)], the activities of CAT, SOD, ARYL, and PON in GSD patients were not different from the controls. GPx activity was decreased in GSD Ia [3.7 (1.8–7.1) U/mL] and GSD III [4.2 (2.2–8.6) U/mL] compared with healthy controls [7.1 (2.9–16.2) U/mL].In conclusion, this study supplied the data for trace elements, their carrier, and antioxidative enzymes in the patients with GSD Ia and III. The trace elements and anti-oxidative enzyme levels in GSD patients failed to explain the atherosclerotic escape phenomenon reported in these patients.  相似文献   
67.
A member of the sirtuin family of NAD+-dependent deacetylases, SIRT3, is located in mammalian mitochondria and is important for regulation of mitochondrial metabolism, cell survival, and longevity. In this study, MRPL10 (mitochondrial ribosomal protein L10) was identified as the major acetylated protein in the mitochondrial ribosome. Ribosome-associated SIRT3 was found to be responsible for deacetylation of MRPL10 in an NAD+-dependent manner. We mapped the acetylated Lys residues by tandem mass spectrometry and determined the role of these residues in acetylation of MRPL10 by site-directed mutagenesis. Furthermore, we observed that the increased acetylation of MRPL10 led to an increase in translational activity of mitochondrial ribosomes in Sirt3−/− mice. In a similar manner, ectopic expression and knockdown of SIRT3 in C2C12 cells resulted in the suppression and enhancement of mitochondrial protein synthesis, respectively. Our findings constitute the first evidence for the regulation of mitochondrial protein synthesis by the reversible acetylation of the mitochondrial ribosome and characterize MRPL10 as a novel substrate of the NAD+-dependent deacetylase, SIRT3.  相似文献   
68.
69.
Gyrodactylus sommervillae n. sp. (Monogenea) is described from the skin, fins and gills of Abramis brama (L.) and Rutilus rutilus (L.) from Blenheim Palace Lake, Oxfordshire, UK. G. sommervillae n. sp. closely resembles G. aphyae Malmberg, 1957, G. kearni Ergens, 1990 and G. lamberti Ergens, 1990 in the approximate shape of the marginal hook sickle and the anchors, but can be separated on the precise shape of the marginal hooks and the ventral bar.  相似文献   
70.
This paper proposes a new metabolic model for acetate uptake by a mixed culture of phosphate- and glycogen-accumulating organisms (PAOs and GAOs) under anaerobic conditions. The model uses variable overall stoichiometry based on the assumption that PAOs may have the ability of using the glyoxylate pathway to produce the required reducing power for polyhydroxyalkonate (PHA) synthesis. The proposed model was tested and verified by experimental results. A sequencing batch reactor system was operated for enhanced biological phosphorus removal (EBPR) with acetate as the sole carbon source at different influent acetate/phosphate ratios. The resulting experimental data supported the validity of the proposed model, indicating the presence of GAOs for all tested HAc/P ratios, especially under P-limiting conditions. Strong agreement is observed between experimental values and model predictions for all model components, namely, PHB production, PHA composition, glycogen utilization, and P release.  相似文献   
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