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It is caused by mutations in dyskerin-encoding genes, telomerase-encoding RNA component genes and reverse transcriptase genes, as well as other uncharacterized genes. There are three inherited forms, including X-linked, autosomal dominant and autosomal recessive inheritance.

先天性角化不良是与编码角化不良蛋白基因、编码端酶的RNA组份基因、编码端酶的逆转录酶基因突变及其他未确认的致病基因突变引起的基因病,其遗传方式有:X-性联隐性遗传、常染色体显性遗传及常染色体隐性遗传。

Based on the polynomial equations deduced from the experimental design, two MCC slurries with different material properties and functionalities can be prepared and co-dried with acetaminophen, which is inherently uncompressible, to produce direct compression formulations.

在了解制程因子对微晶纤维素的整体影响性后,进一步应用上述实验设计法所得到的多项式方程式,制备具不同材料性质与功能性的微晶纤维素湿,利用此湿以共同乾燥法,修饰本身不具压锭性的acetaminophen,使之成为直打药物。

Low-molecular-weight EC was attained with ethylcellulose being degraded by 34% nitric acid; Fourier transform infrared spectroscopy, 13C-NMR,element analysis confirmed that the basic structure and major properties of low-molecular-weight EC maintained agreement with those of undegraded EC except that the polymerization degree of EC decreased.

乙基纤维素被 34%硝酸降解可以得到低分子量的乙基纤维素;FTIR,1 3C- NMR,元素分析,X-射线衍射分析证实在硝酸降解过程中,除了聚合度和结晶度降低之外,乙基纤维素的基本特性和基本结构没有变化;EC分子量的大小影响纳米微球的径大小、径分布和对药物的包覆效率。

Small-scale fertilizer granulator is mainly composed by motor, making grains disc , gearbox , underbed, main shaft bearing , regulate device and rotational speed test system ,etc.

小型肥料制机主要由电动机、制圆盘、变速箱、底架、主轴轴承、调节装置和转速测试系统等组成。

The recombinant plasmid DNA of PrM-E gene but not unmethylated CpG was capable of inducting antibodies against D2-43 virus.

我国登革2型病毒PrM-E基因重组质DNA具有一定的免疫原性。在本实验条件下,含非甲基化细菌性CpG的pUC19质DNA,对PrM-E基因的DNA免疫效果没有促进作用。

And hides without hiding behind an unminding cloud.

我们叫它一沙。但它不叫自己或沙。

The unsmoothed characteristics of some living beings in soil have wear reduction and wear resistance function . The research benefits the revelation that a tungsten carbide reinforced Cu-base alloy wear-resistant composite coating was produced on mild steel by spark plasma sintering.Under the condition of two bodies abrasive wear ,the coating with the structure that hight hardness and good wear resistance WC particles embedding in tough fine Cu-base alloy matrix resist the abrasion wear and manifest the good wear reduction and wear resistance properties that the unsmoothed bodysurface of some living beings in soil have.

本研究受到某些土壤动物体表非光滑几何特征具有减磨、耐磨特性的启示,应用放电等离子烧结工艺,选取具有优良塑性的Cu基合金作为复合层基体,硬质点WC颗作为增强相,在碳钢表面形成WC\ Cu基合金耐磨复合层,在两体静载荷磨磨损条件下,高硬度、耐磨WC颗镶嵌在高塑性的Cu基合金基体中,共同抵抗磨的磨削,表现出某些土壤动物体表非光滑几何特征所具备的优良减磨、耐磨特性。

I not only thought these the pure Productions of Providence for my Support, but not doubting, but that there was more in the Place, I went all over that Part of the Island, where I had been before, peering in every Corner, and under every Rock, to see for more of it, but I could not find any; at last it occur'd to my Thoughts, that I had shook a Bag of Chickens Meat out in that Place, and then the Wonder began to cease; and I must confess, my religious Thankfulness to God's Providence began to abate too upon the Discovering that all this was nothing but what was common; tho' I ought to have been as thankful for so strange and unforseen Providence, as if it had been miraculous; for it was really the Work of Providence as to me, that should order or appoint, that 10 or 12 Grains of Corn should remain unspoil'd,(when the Rats had destroy'd all the rest,) as if it had been dropt from Heaven; as also, that I should throw it out in that particular Place, where it being in the Shade of a high Rock, it sprang up immediately; whereas, if I had thrown it anywhere else, at that Time, it had been burnt up and destroy'd.

当时,我不仅认为这些谷类都是老天为了让我活命而赐给我的,并且还相信岛上其他地方一定还有。于是,我在岛上搜遍了我曾经到过的地方,每个角落,每块岩石边我都查看了一遍,想找到麦穗和稻秆,可是,再也找不到了。最后,我终于想起,我曾经有一只放鸡饲料的袋子,我把里面剩下的谷壳抖到了岩壁下。这一想,我惊异的心情一扫而光。老实说,我认为这一切都是极其平常的事,所以我对上帝的感恩之情也随之减退了。然而,对发生这样的奇迹,对意料之外的天意,我还是应该感恩戴德的。老鼠吃掉了绝大部分谷,而仅存的十几颗竟然没有坏掉,仿佛从天上掉下来似的,发生这样的奇迹难道不是天意又是什么呢?再说,我把这十几颗谷不扔在其他地方,恰恰扔在岩壁下,因而遮住了太阳,使其很快长了出来;如果丢在别处,肯定早就给太阳晒死了,这难道不是天意吗?

The operation methods and operation main points of SQL 950 submerged granulator were introduced including the start and stop operation of the granulator, the flow distribution of the three streams of water, the operation cycle of the desalinized water plate exchanger, the easy occlusion of the nozzle, the chip stacking of the long chip accumulator and clogged with conveying waterpipe, the easy overload and untight seal of the centrifugal drier.

介绍了SQL 950型水下切机的操作方法及操作要点,包括切的开机操作,停机操作,3股水的流量分配,脱盐水板式换热器运行周期,喷嘴易堵塞,长料收集器子堆积堵塞输送水管,离心干燥机易过载和密封不严的处理。

Fig. 1 Changes of yeld, number of pods, number of seeds, and 100-seed weight with year of released cultivars.

图1 产量﹑单株荚数﹑单株数和百重随育成年代的变化。

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According to the clear water experiment, aeration performance of the new equipment is good with high total oxygen transfer coefficient and oxygen utilization ratio.

曝气设备的动力效率在叶轮转速为120rpm~150rpm时取得最大值,此时氧利用率和充氧能力也具有较高值。

The environmental stability of that world - including its crushing pressures and icy darkness - means that some of its most famous inhabitants have survived for eons as evolutionary throwbacks, their bodies undergoing little change.

稳定的海底环境─包括能把人压扁的压力和冰冷的黑暗─意谓海底某些最知名的栖居生物已以演化返祖的样态活了万世,形体几无变化。

When I was in school, the rabbi explained everythingin the Bible two different ways.

当我上学的时候,老师解释《圣经》用两种不同的方法。