148893-10-1|2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯|HATU，技术参数

1.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯产品物理参数：

2.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯同类产品列表：

3.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯物理化学性质：

4.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯急救措施：

吸入： 将受害者移到新鲜空气处，保持呼吸通畅，休息。若感不适请求医/就诊。
皮肤接触： 立即去除/脱掉所有被污染的衣物。用大量肥皂和水轻轻洗。
若皮肤刺激或发生皮疹：求医/就诊。
眼睛接触：用水小心清洗几分钟。如果方便，易操作，摘除隐形眼镜。继续清洗。
如果眼睛刺激：求医/就诊。
食入： 若感不适，求医/就诊。漱口。
紧急救助者的防护：救援者需要穿戴个人防护用品，比如橡胶手套和气密性护目镜。

5.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯消防措施：

合适的灭火剂：干粉，泡沫，雾状水，二氧化碳
特殊危险性：小心，燃烧或高温下可能分解产生毒烟。
特定方法：从上风处灭火，根据周围环境选择合适的灭火方法。
非相关人员应该撤离至安全地方。
周围一旦着火：如果安全，移去可移动容器。
消防员的特殊防护用具：灭火时，一定要穿戴个人防护用品。

6.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯泄漏应急处理：

个人防护措施，防护用具， 使用个人防护用品。远离溢出物/泄露处并处在上风处。
紧急措施：泄露区应该用安全带等圈起来，控制非相关人员进入。
环保措施：防止进入下水道。
控制和清洗的方法和材料：清扫收集粉尘，封入密闭容器。注意切勿分散。附着物或收集物应该立即根据合适的法律法规处置。

7.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯操作处置与储存：

① 处理：
技术措施：在通风良好处进行处理。穿戴合适的防护用具。防止粉尘扩散。处理后彻底清洗双手和脸。
注意事项：如果粉尘或浮质产生，使用局部排气。
操作处置注意事项：避免接触皮肤、眼睛和衣物。
② 贮存：
储存条件：保持容器密闭。冷藏储存。
存放于惰性气体环境中。
③ 防湿：
远离不相容的材料比如氧化剂存放。
④ 热敏, 潮敏：
包装材料：依据法律。

8.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯毒性和生态：

生态学数据:

其它有害作用：通常对水是稍微危害的，若无政府许可，勿将材料排入周围环境。

9.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯文献：

Antisense Oligonucleotide-mediated Suppression of Muscle Glycogen Synthase 1 Synthesis as an Approach for Substrate Reduction Therapy of Pompe Disease.

Nicholas P Clayton, Carol A Nelson, Timothy Weeden, Kristin M Taylor, Rodney J Moreland, Ronald K Scheule, Lucy Phillips, Andrew J Leger, Seng H Cheng, Bruce M Wentworth

文献索引：Mol. Ther. Nucleic Acids 3 , e206, (2014)

全文：HTML全文

摘要

Pompe disease is an autosomal recessive disorder caused by a deficiency of acid α-glucosidase (GAA; EC 3.2.1.20) and the resultant progressive lysosomal accumulation of glycogen in skeletal and cardiac muscles. Enzyme replacement therapy using recombinant human GAA (rhGAA) has proven beneficial in addressing several aspects of the disease such as cardiomyopathy and aberrant motor function. However, residual muscle weakness, hearing loss, and the risks of arrhythmias and osteopenia persist despite enzyme therapy. Here, we evaluated the relative merits of substrate reduction therapy (by inhibiting glycogen synthesis) as a potential adjuvant strategy. A phosphorodiamidate morpholino oligonucleotide (PMO) designed to invoke exon skipping and premature stop codon usage in the transcript for muscle specific glycogen synthase (Gys1) was identified and conjugated to a cell penetrating peptide (GS-PPMO) to facilitate PMO delivery to muscle. GS-PPMO systemic administration to Pompe mice led to a dose-dependent decrease in glycogen synthase transcripts in the quadriceps, and the diaphragm but not the liver. An mRNA response in the heart was seen only at the higher dose tested. Associated with these decreases in transcript levels were correspondingly lower tissue levels of muscle specific glycogen synthase and activity. Importantly, these reductions resulted in significant decreases in the aberrant accumulation of lysosomal glycogen in the quadriceps, diaphragm, and heart of Pompe mice. Treatment was without any overt toxicity, supporting the notion that substrate reduction by GS-PPMO-mediated inhibition of muscle specific glycogen synthase represents a viable therapeutic strategy for Pompe disease after further development.

10.2-(7-氮杂-1H-苯并三氮唑-1-基)-1,1,3,3-四甲基脲六氟磷酸酯英文别名：