查询词典 Pompe

With small and medium enterprises related literature like Pompe and Bilderbeek (2005), Bai Qin Yuan (2003), Zhang Kuohao (2004) and so on the financial prewarning research the model in view of the discussion small and medium enterprises , many but the natrium has not joined a society take the financial factor as the research variable the influence enterprise survival development the non-financial factor.

与中小企业有关文献如Pompe and Bilderbeek(2005)、白钦元(2003)、张国浩(2004)等针对探讨中小企业财务危机预警模式之研究，多以财务比率为研究变数而未纳入会影响企业生存发展之非财务因素。

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。

The witness also told the jury at the Royal Courts of Justice in London that he saw a paparazzo fighting with a member of the public who was trying to stop him taking pictures in the minutes before the emergency services arrived.

他还告诉在伦敦皇家法庭的陪审团，在急救服务到来之前，他当时看见一个狗仔队正和一群阻止他拍照的人打架。

The entire N／C program on a tape is made up of an accumulation of these successive data blocks.

纸带上的整个数控程序由这些连续数据单元连接而成。