2. Metabolic Enzyme/Protease
  3. Glycosidase
  4. 5-C-phenethyl-DNJ hydrochloride

5-C-phenethyl-DNJ (hydrochloride) 是一种具有口服活性的酸性 α-葡萄糖苷酶 (GAA) 激活剂。5-C-phenethyl-DNJ (hydrochloride) 可提高多种组织中的 GAA 活性，尤其在膈肌中。5-C-phenethyl-DNJ (hydrochloride) 可用于代谢疾病的研究，如庞贝病。

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5-C-phenethyl-DNJ hydrochloride

5-C-phenethyl-DNJ hydrochloride Chemical Structure

CAS No. : 2763641-30-9

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Other Forms of 5-C-phenethyl-DNJ hydrochloride:

5-C-phenethyl-DNJ hydrochloride 相关抗体

Top Publications Citing Use of Products

  • 生物活性

  • 纯度 & 产品资料

  • 参考文献

生物活性

5-C-phenethyl-DNJ (hydrochloride) is an orally active acid α-glucosidase (GAA) activator. 5-C-phenethyl-DNJ (hydrochloride) can increase GAA activity in various tissues, particularly in the diaphragm. 5-C-phenethyl-DNJ (hydrochloride) can be used for the research of metabolic disease, such as Pompe disease[1].

IC50 & Target[1]

α‑glucosidase

 

体外研究
(In Vitro)

5-C-phenethyl-DNJ (hydrochloride) (Compound 4c) (30 mins) 可特异性抑制重组人酸性 α-葡萄糖苷酶 (rhGAA) 的活性，其 IC50 值为 0.81 μM[1]。
5-C-phenethyl-DNJ (hydrochloride) (100 μM) 在中性 pH (7.4) 条件下能显著提高 rhGAA 的稳定性，并维持 rhGAA 在血浆和非溶酶体细胞区室中的结构稳定性[1]。
5-C-phenethyl-DNJ (hydrochloride) (0.1-100 μM, 30-60 mins) 对其他人类糖苷酶表现出优异的选择性，对 GANAB、GBA1 和 GBA2 的 IC50 值均大于 100 μM[1]。
5-C-phenethyl-DNJ (hydrochloride) (5-20 μM, 24 h) 能显著恢复携带不同 GAA 突变的庞贝病成纤维细胞中内源性 GAA 的活性[1]。
5-C-phenethyl-DNJ (hydrochloride) (20 μM, 24 h) 可增强庞贝病成纤维细胞中 rhGAA 的活性及其向溶酶体的递送[1]。

MCE has not independently confirmed the accuracy of these methods. They are for reference only.

5-C-phenethyl-DNJ hydrochloride 相关抗体:
体内研究
(In Vivo)

5-C-phenethyl-DNJ (hydrochloride) (Compound 4c) (5 mg/kg，口服，每日一次，连续 4 天) 显著增强了 GAA 敲除小鼠组织中 rhGAA 的活性[1]。

MCE has not independently confirmed the accuracy of these methods. They are for reference only.

Animal Model: GAA-KO mice models[1]
Dosage: 5 mg/kg, companied with 40 mg/kg rhGAA i.v. at day 3
Administration: Orally gavage
Result: Increased the GAA activity by 2 folds in diaphragm (a key tissue for respiratory function).
Increased the GAA activity by 1.5 folds in heart and quadriceps.
Showed no significant difference in liver activity.
分子量

303.78

Formula

C14H22ClNO4
CAS 号
运输条件

Room temperature in continental US; may vary elsewhere.
储存方式

Please store the product under the recommended conditions in the Certificate of Analysis.
纯度 & 产品资料
参考文献

5-C-phenethyl-DNJ hydrochloride 相关分类

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  • Do most proteins show cross-species activity?

    Species cross-reactivity must be investigated individually for each product. Many human cytokines will produce a nice response in mouse cell lines, and many mouse proteins will show activity on human cells. Other proteins may have a lower specific activity when used in the opposite species.

Keywords:

5-C-phenethyl-DNJ2763641-30-9Glycosidaseacid α-glucosidaseGAAPompe diseaseFibroblastsInhibitorinhibitorinhibit

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