中文名 | 二甲基磺酸庚胺 |
英文名 | Heptamidine dimethanesulfonate |
别名 | 二甲基磺酸庚胺 4,4'-(庚烷-1,7-二基双(氧基))二苯甲脒 二甲磺酸盐 化合物HEPTAMIDINE DIMETHANESULFONATE |
英文别名 | SBi4211 dimethanesulfonate Heptamidine dimethanesulfote Heptamidine dimethanesulfonate 4,4'-(Heptane-1,7-diylbis(oxy))dibenzimidamide dimethanesulfonate |
CAS | 161374-55-6 |
化学式 | C22H32N4O5S |
分子量 | 464.58 |
存储条件 | 2-8°C,密封,干燥 |
外观 | 固体 |
体外研究 | Heptamidine is a Pentamidine-S100B complex, two molecules of pentamidine bind per monomer of S100B, which performs to be an inhibitor for S100B.Heptamidine (20 μM) does not decrease CUG levelssignificantly when compares to Propamidine and Pentamidine, and exhibits cytotoxic at concentrations above 17.5 μM in HeLa cells expressing 960 CUG repeats.Heptamidine rescues mis-splicing of minigene reporters in a HeLa cell DM1 model with an EC 50 value of 15 μM. |
体内研究 | Heptamidine (intraperitoneal injection; 20 or 30 mg/kg; 7 days) causes a dose-dependent reduction of exon 7a inclusion in HSA LRmice, returning to wild type levels (6±1%) when at 20 mg/kg dose, the myotonia is reduced from grade 3 to grade 1 (occasional myotonic discharge) or grade 0 at both 20 or 30 mg/kg. Animal Model: HomozygousHSALRtransgenic mice in line 20b (FVB inbred background)with a Myotonic dystrophy (DM) mouse model Dosage: 20 or 30 mg/kg Administration: Intraperitoneal injection; 7 days; once daily Result: Reversed splicing defects and rescues myotonia in a DM mouse model. |
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