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71.
Glycoproteinoses, Pompe disease, and sialic acid storage diseases are characterized by a massive accumulation of unprocessed oligosaccharides and/or glycoconjugates in urine. The identification of these glycocompounds is essential for a proper diagnosis. In this study, we investigated the potential of MALDI‐TOF‐MS to identify glycocompounds present in urine from patients with different inborn errors of glycan metabolism. Urinary glycocompounds were permethylated, and analyzed using GC‐MS and MALDI‐TOF‐MS. In order to confirm tentative assignments, a second aliquot of urine was purified on a C18 Sep‐Pak cartridge and glycocompounds were desalted on a column of nonporous graphitized carbon. The glycocompounds were then sequentially on‐plate digested using an array of exoglycosidases. A range of disease‐specific oligosaccharides as well as glycopeptides was identified for all oligosacchariduria models. In addition, free sialic acid accumulated in urine from a patient suffering from French‐type sialuria, has been detected by a GC‐MS approach, which could be applied to other sialic acid storage diseases. This procedure is simple, and can be performed in few simple steps in less than 24 h. This current method can be applied for newborn screening for other inherited metabolic diseases as well as for assessing treatments in clinical trials. 相似文献
72.
73.
Extracts of maleMamestra brassicae (L.) hairpencils were analyzed by capillary gas chromatography (GC) and by GC-mass spectrometry (GC-MS). The extracts were found to consist of six components. Benzaldehyde, 2-methylpropanoic acid, 2-methylbutanoic acid, and phenol were present in the extracts as well as the previously identified benzyl alcohol and phenyl ethanol. The two major components were 2-phenylethanol and 2-methylbutanoic acid. They represented, respectively, 74% and 12.5% of the total blend in 3-day-old male extracts. Electroantennograms were recorded on male and female antennae in response to stimulation by hairpencil compounds. Male and female antennae responded to each chemical but the female responses were significantly higher than those of the males. 相似文献
74.
To investigate the effects of OA on the portland cement using oleic acid (OA) as grinding aid and the effects of OA on the portland cement, we characterized the different perfermances of the cement, discussed the characteristics of properties. The results reveal that the OA can reduce the water requirement of normal consistency. With the content of OA added, setting time was extended. The OA can significantly improve the rheological properties of cement-based materials, while the compressive strength decreases. 相似文献
75.
应用Menke条件,在改性高岭土固体酸催化下,甲苯能被浓硝酸对位选择性硝化,表现在对邻硝化产物比例达到1.5。 相似文献
76.
77.
世界耐除草剂转基因作物的发展及问题 总被引:4,自引:0,他引:4
介绍了耐除草剂转基因作物的发展概况及存在问题,以及它们的解决措施。 相似文献
78.
叶树滋 《硫磷设计与粉体工程》2003,(5):10-13
针对采用水洗净化工艺的某80kt/a硫铁矿制酸装置的扩建设计,介绍了其中主要改扩建工段及设备采用的新技术措施,设计中对原料工段、沸腾炉、文氏管、泡沫塔、转化器、换热器等进行了改进,满足了厂方两期建设产量不同的要求。 相似文献
79.
研究了铜与1-(2-吡啶偶氮)-2-萘酚(PAN)的显色反应,建立了测定硫酸阳极氧化液中铜的新方法。以TritonX-100为增溶增敏剂,明胶作稳定保护剂,在酸性条件下Cu-PAN络合物稳定保持在水相中,直接进行光度分析。该络合物在558nm处有最大吸收峰,其表观摩尔吸光系数ε=2.25×104L.mol-1.cm-1。方法检出限为0.028μg/mL,线性范围为0~3.2μg/mL,用于测定硫酸阳极氧化液中的铜,加标回收率为98.2%~105.6%,相对标准偏差≤0.34%。 相似文献
80.