查询词典 Pompe

Aberrant activities have been implicated in diseases such as diabetes and Pompe disease.

alpha；-葡萄糖苷酶的活性异常可能与糖尿病、庞佩氏症等疾病有关。

Pompe's disease affects mainly children and can prove fatal by causing heart and lung failure.

庞贝氏症主要是发生在小孩身上，会因为心脏、呼吸衰竭死亡。

Late-onset Pompe''s disease is characterized by progressive muscle weakness and loss of respiratory function, leading to early death.

晚发式蓬佩病的特性是进行性肌无力和呼吸功能丧失，从而导致早期死亡。

Methods: We first analyzed phenotype and genotypes of Pompe disease in Taiwan, and explored factors which may affect the outcome.

我们首先分析台湾庞贝氏症患者之表现型、基因型、以及个案之分布，以及是否有导致治疗失败之因子。

Enzymatic diagnosis of Pompe disease is hampered by the presence of several isoenzymes in whole blood, of which the neutrophil enzyme maltase-glucoamylase is of particular concern due to its low pH optimum.

酶法诊断庞佩病是受到在场的几个同工酶全血，而中性酶麦芽糖-糖化酶是特别关注，由於其低ph最优。

An autosomal recessive genetic condition, Pompe's disease affects 1 in 40,000 individuals.

该试验已经於12月6日线上发表，并预计於2007年1月9日发表於神经学期刊。

In late-onset Pompe disease, most of the symptoms cannot be reversed by treatment.

婴儿型庞贝氏症患者经由酵素补充疗法，可有效延长性命，恢复心脏正常大小，但是大部分患者的运动及呼吸功能无法恢复正常；晚发型庞贝氏症治疗后，其症状之可逆性也很有限。

Background: Pompe disease is due to a deficiency of lysosomal acid alpha glucosidase, and currently an enzyme replacement therapy has been developed.

中文摘要背景：庞贝氏症是一种因为溶小体缺乏酸性醣苷酶无法分解肝醣所引起之疾病。

The biotechnology company Genzyme spent five hundred million dollars developing the drug Myozyme, which is intended for a condition, Pompe disease, that afflicts fewer than ten thousand people worldwide.

生物技术公司健赞投入5亿美元研发了一种治疗庞贝氏症的治疗药Myozyme，这种疾病的患者在全世界不超过1万名。

The medication developed by the Academia Sinica team is the only approved treatment for Pompe's disease available, giving children who originally thought they would never grow up, another chance at life.

全球唯一也是第一个治疗庞贝氏症的药物，核准上市，更奠定治愈其他肌肉病变的基础，这也给了更多原本以为长不大的孩子，一线希望。

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。