| Bioactivity | ARGX-119 is a first-in-class humanized agonist monoclonal antibody specific for muscle-specific kinase (MuSK). ARGX-119 can be used for the study of neuromuscular diseases. The isotype control for ARGX-119 can refer to Human IgG1 lambda1, Isotype Control (HY-P99992)[1]. |
| Invitro | ARGX-119 与人类、非人类灵长类动物、大鼠和小鼠 MuSK 的 Frizzled 样结构域具有高亲和力结合，没有脱靶结合，适合临床开发。在 Fc 区域内，ARGX-119 含有 L234A 和 L235A 突变，可削弱潜在的免疫激活效应功能。其作用方式是激活 MuSK，而不会干扰其天然配体神经 Agrin，并以剂量依赖性方式聚集乙酰胆碱受体，从而稳定神经肌肉功能[1]。 MCE has not independently confirmed the accuracy of these methods. They are for reference only. 0 --> ARGX-119 相关抗体: |
| In Vivo | 在 DOK7 先天性肌无力 (CM) 小鼠模型中，ARGX-119 (20 mg/kg) 可通过剂量依赖性方式恢复神经肌肉功能并减少肌肉无力和易疲劳，从而预防早期出生后死亡并逆转成年 Dok7 CM 小鼠的疾病复发[1]。 MCE has not independently confirmed the accuracy of these methods. They are for reference only. |
| CAS | 2966023-63-0 |
| Storage | Please store the product under the recommended conditions in the Certificate of Analysis. |
| Reference | [1]. Vanhauwaert R, et al. ARGX-119 is an agonist antibody for human MuSK that reverses disease relapse in a mouse model of congenital myasthenic syndrome. Sci Transl Med. 2024 Sep 18;16(765):eado7189. |