查询词典 Pompe

We conducted a randomized, placebo-controlled trial of alglucosidase alfa, a recombinant human GAA, for the treatment of late-onset Pompe''s disease.

咱们进行了一项用阿葡糖苷酶α治疗晚发式蓬佩病的RAND、慰藉剂对于照试验。

Background Pompe''s disease is a metabolic myopathy caused by a deficiency of acid alpha glucosidase, an enzyme that degrades lysosomal glycogen.

配景 蓬佩病是一种由酸性α葡萄糖苷酶（GAA，一种降解溶酶体肝糖的酶）缺乏引起的代谢性肌病。

ALPHA POMPE SPA presents the new line of pneumatic rack ＆amp; pinion Stainless Steel actuators, particularly engineered for corrosive environements or Sanitary.

阿尔法庞佩集介绍了新的气动齿条＆小齿轮传动装置，不锈钢，特别是专为腐蚀性 environements 或卫生。

Pompe's disease is a rare hereditary condition that causes an enzyme deficiency, which in turn leads to the buildup of glycogen, a stored form of sugar used for energy. The glycogen buildup causes progressive muscle weakness throughout the body, particularly in the heart, skeletal muscles, liver, and nervous system. In extreme cases, children can also die of heart failure.

庞贝氏症是一种遗传的罕见疾病，因为缺乏体内的酵素，导致肝醣无法分解堆积，因肝醣无法分解堆积造成心脏肥大、肌肉无力，肝脏和神经系统衰弱，在ㄧ些更极端的病例中，最后就因为心脏衰竭死亡。

In order to realize the etiology, I agree that the Department of Medical Genetics NTUH, or their consulting institutes, can use the DNA extracted from either blood or tissues of my child and the parents forthe genetic analysis of Pompe disease.

今同意台大医院基因医学部及其委托之检验机构，采用小孩及父母亲之血液或其他组织检体中之DNA，进行庞贝氏症之基因分析，以了解其原因。

Through newborn screening, we also identified prominent GAA gene variations in our population, including the "pseudodeficiency" allele and alleles which may modify the clinical manifestations of Pompe disease.

这些变异型会使得酸性醣苷酶活性偏低，我们目前不能排除这些基因变异型是否参与庞贝氏症临床表现之决定。

On June 18, the FDA granted orphan drug designation to 1-deoxynojirimycin hydrochloride for the treatment of Pompe disease, an inherited lysosomal storage disorder caused by a mutation that alters the structure and stability of α-glucosidase, an enzyme responsible for the breakdown of glycogen.

6月18日，FDA批准孤儿药盐酸1-脱氧野尻霉素（Amicus Therapeutics， Inc）治疗蓬佩病。该疾病是一种遗传性溶体缺陷症，因突变导致破坏糖原的α糖苷酶的结构和稳定性改变。

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。