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91.
We report a new form of congenital muscular dystrophy (CMD) in 4 patients from three unrelated families with probable autosomal-recessive inheritance. All patients had the clinical characteristics of merosin-positive congenital muscular dystrophy, but had marked mental retardation. The disease was slowly progressive and 1 patient died from dilated cardiomyopathy at the age of 13 years. In addition to dystrophic changes with necrosis and regeneration in muscle, the most striking finding was mitochondrial depletion in the center of the sarcoplasm. Mitochondria at the periphery of fibers were markedly enlarged ("megaconial" appearance) with complicated cristae, and contained a normal amount of mitochondrial DNA by in situ hybridization. Mitochondrial enlargement may represent functional compensation for mitochondrial depletion in the central sarcoplasm, where myofibrillar degeneration occurred. 相似文献
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93.
Settivari RS Spain JN Ellersieck MR Byatt JC Collier RJ Spiers DE 《Journal of dairy science》2007,90(3):1265-1280
The responses of lactating Holstein cows to daily administration of bovine somatotropin (bST) were measured at thermoneutrality (Tn) and under both constant and cycled heat-stress conditions to determine the relationship between thermal status and bST-induced shifts in milk production. All tests included a 5-d acclimation period at Tn (18°C), followed by a 2-d increase in ambient temperature to 28.5°C. After d 3, ambient temperature was cycled between 28.5 (day) and 25.5°C (night) for 4 d. Daily injections with either 31 mg of bST or saline began on d 1 of the experiment. Milk production, feed intake, and respiratory rate (RR) were measured daily. Intraperitoneal, telemetric temperature transmitters were used for a continuous measure of core body temperature (Tcore). Blood samples were collected during each phase to evaluate the changes in serum chemistry in response to bST and heat stress. Following a 15-d recovery, cows were switched across injection treatments and the study was repeated. Milk production decreased by ∼18.4% below the initial yield at Tn by the end of 7 d of heat challenge. Although a reduction in milk production occurred during heat stress in both groups, milk production was higher in bST-treated cows compared with control cows during periods of constant and cyclic heat. Likewise, bST treatment during the entire period increased the milk-to-feed ratio over the control level by ∼11.3%. Plasma insulin-like growth factor 1 and serum nonesterified fatty acids accompanied the increased growth hormone level with bST treatment (∼122.0 and 88.8%, respectively), whereas plasma urea nitrogen was reduced by ∼13.3% to reflect the shift to lipid metabolism. There was no difference in Tcore of the treatment and control groups at Tn. Both bST and control cows increased RR and Tcore above the Tn level by ∼94.8 and 2.9%, respectively, during constant heat, with a greater increase in Tcore of bST-treated compared with control cows (∼0.6%). The increase in RR during heat stress preceded Tcore by 1 d for both groups. During cyclic heat, Tcore decreased by ∼0.4% compared with constant heat in both the control and bST-treated groups. Bovine somatotropin treatment increased milk production similarly during the Tn and heat-stress periods, ∼8.3% over the control; however, the bST-induced increase in milk-to-feed ratio was greatest during the continuous and cyclic heat-stress phases, ∼16.2%. This increase occurred together with the elevation in Tcore. 相似文献
94.
A. S. Bedekar J. J. Feng S. Krishnamoorthy - S. Krishnamoorthy is currently with Baxter Healthcare Corporation Round Lake IL. K. G. Lim G. T. R. Palmore S. Sundaram 《Chemical Engineering Communications》2008,195(3):256-266
Biofuel cells are devices that use biocatalysts (enzymes or microbes) to convert biochemical energy directly into electrical energy. Microfluidic biofuel cells exploit the lack of active mixing at microscale dimensions to eliminate the use of proton exchange membranes that separate anolyte and catholyte streams. Simulation of this system, by solving the governing 3-D conservation equations (flow, species transport), reveals that oxygen availability limits the performance of the cathode. An exponential decay in the availability of oxygen at the cathode is observed along the length of the microchannel, indicating that increasing the number of electrode pairs reduces the overall current density. This conclusion is consistent with experimental observations. Increasing electrolyte flow rates can reduce the mass transport limitations by decreasing the diffusion boundary-layer thickness, but disparity between the flow rates of the anolyte and catholyte can induce wastage of dissolved oxygen. 相似文献
95.
The physicochemical properties and antioxidant properties of bokbunja, a Korean black raspberry, were clarified and compared with blueberry, cranberry, raspberry, and strawberry. The amount of soluble solids was significantly (p<0.05) lower in bokbunja than in other berries, whereas the pH of bokbunja was significantly (p<0.05) higher. Analysis of the total phenolic, flavonoid, and anthocyanin contents found that bokbunja had the highest, while the others were ranked in the order of raspberry>blueberry>cranberry>strawberry. The content of ellagic acid (147.3 mg/100 g of FW) also was highest in bokbunja. The antioxidant activities of bokbunja, assessed based on DPPH, ABTS, and ferric reducing antioxidant power (FRAP) assays, were higher than for other berries. Bokbunja is a healthier antioxidant source than other berries. 相似文献
96.
Bhushan B Halasz A Spain J Thiboutot S Ampleman G Hawari J 《Environmental science & technology》2002,36(14):3104-3108
Hexahydro-1,3,5-trinitro-1,3,5-triazine (RDX) can be efficiently mineralized with anaerobic domestic sludge, but the initial enzymatic processes involved in its transformation are unknown. To test the hypothesis that the initial reaction involves reduction of nitro group(s), we designed experiments to test the ability of a nitrate reductase (EC 1.6.6.2) to catalyze the initial reaction leading to ring cleavage and subsequent decomposition. A nitrate reductase from Aspergillus niger catalyzed the biotransformation of RDX most effectively at pH 7.0 and 30 degrees C under anaerobic conditions using NADPH as electron donor. LC/MS (ES-) chromatograms showed the formation of hexahydro-1-nitroso-3,5-dinitro-1,3,5-triazine (MNX) and methylenedinitramine as key initial products of RDX, but neither the dinitroso neither (DNX) nor trinitroso (TNX) derivatives were observed. None of the above detected products persisted, and their disappearance was accompanied by the accumulation of nitrous oxide (N20), formaldehyde (HCHO), and ammonium ion (NH4+). Stoichiometric studies showed that three NADPH molecules were consumed, and one molecule of methylenedinitramine was produced per RDX molecule. The carbon and nitrogen mass balances were 96.14% and 82.10%, respectively. The stoichiometries and mass balance measurements supported a mechanism involving initial transformation of RDX to MNX via a two-electron reduction mechanism. Subsequent reduction of MNX followed by rapid ring cleavage gave methylenedinitramine which in turn decomposed in water to produce quantitatively N2O and HCHO. The results clearly indicate that an initial reduction of a nitro group by nitrate reductase is sufficient for the decomposition of RDX. 相似文献
97.
四、第一代表面工程方法 1.固态淬火(硬化) 加热的各项工艺参数、材料的初始组织和机件的具体几何形状,都对材料的组织和性能、淬硬的深度和机件的表面质量有很大的影响。在我们的试验研究中,对各种不同的结构钢和工具钢以及某些铁进行了研究,采用的加热方式为将需要淬硬的整个表面同时加热等。对于退火状态的和整体淬硬的多,硬化度分别达到3.6~3.7和1.5~1.7。钢的硬度达到HRC65~68,显微硬度为10~11GPa,硬化的深度为 1.5-2.0mm。 在各种钢中,会产生残余的压缩应力,还会形成细粒组织,并… 相似文献
98.
99.
100.
AJ Louw IL Carstens JE Hartshorne TJ van Wyk Kotze 《Canadian Metallurgical Quarterly》1993,48(4):183-187
With the anticipated increase in the aged adult population and the associated gingival recession, the prevalence of root caries is expected to increase. The purpose of this study was to determine the experience and distribution of root caries in a group of aged adults living in Kayelitsha. All non-institutionalized elderly black adults participating in a community geriatric programme were examined. Root caries was recorded using visual and tactile criteria and expressed as the root caries index (RCI) rate. The mean age of the subjects was 65.2 years, the mean number of teeth present was 17.3 and the mean RCI rate was 2.2 per cent. All subjects had gingival recession while only 23.8 per cent had root caries. No surfaces with restored root caries lesions were found. In the maxilla the highest RCI rate was observed on the interproximal surfaces of the posterior teeth (4.4 per cent) but in the mandible the buccal surfaces of the posterior teeth had the highest RCI rate (4.2 per cent). In both the maxilla and the mandible the lingual surfaces of the anterior teeth showed no root caries. Maxillary teeth did not have a significantly higher root caries attack rate than mandibular teeth. Root caries does not appear to be a public health problem in the sampled population. 相似文献