Connected a segment of CMV enhancer to the front of MyoG gene promoter and then constructed the corresponding dual luciferase expression vector pGL3-CMV-MyoGpro. We set four eukaryotic expression vectors including pGL...Connected a segment of CMV enhancer to the front of MyoG gene promoter and then constructed the corresponding dual luciferase expression vector pGL3-CMV-MyoGpro. We set four eukaryotic expression vectors including pGL3-CMV, pGL3MyoGpro, pGL3-CMV-MyoGpro, and pGL3-Basic which contained CMV promoter, MyoG promoter, CMV-MyoG synthesis promoter, and a promoterless negative control, respectively. Then the four vectors and internal control Renilla luciferase report gene vector phRL-TK were transfected into bovine skeletal muscle satellite cells, mouse C2C12 cells and bovine fetal fibroblast cells to detect the promoter activity with dual luciferase report system. The results showed that CMV enhancer could significantly improve the transcription activity of bovine MyoG gene promoter in muscle satellite cells and mouse C2C12 cells, and it had certain specificity. This study provided experimental materials for increasing the high expression of exogenous gene in bovine muscle cells, and also laid the molecular theoretical basis for obtaining the high specific promoter of bovine muscle and the transgenic beef cattle.展开更多
Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-o...Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-ocular, orofacial and limb muscles. The clinical presentation of MuSK MG can vary, which may delay diagnostic procedures. We present a patient who initially presented with severe weight loss and slowly progressive developing neck extensor weakness and diplopia. A single fiber EMG led to the diagnosis MG and antibodies directed towards MuSK were detected. He was treated with prednisone and the steroid sparing agent azathioprine after which he made full recovery.展开更多
基金Supported by the Major Special Projects of New Product Training of Transgenic Organisms(zx080072008-2008)
文摘Connected a segment of CMV enhancer to the front of MyoG gene promoter and then constructed the corresponding dual luciferase expression vector pGL3-CMV-MyoGpro. We set four eukaryotic expression vectors including pGL3-CMV, pGL3MyoGpro, pGL3-CMV-MyoGpro, and pGL3-Basic which contained CMV promoter, MyoG promoter, CMV-MyoG synthesis promoter, and a promoterless negative control, respectively. Then the four vectors and internal control Renilla luciferase report gene vector phRL-TK were transfected into bovine skeletal muscle satellite cells, mouse C2C12 cells and bovine fetal fibroblast cells to detect the promoter activity with dual luciferase report system. The results showed that CMV enhancer could significantly improve the transcription activity of bovine MyoG gene promoter in muscle satellite cells and mouse C2C12 cells, and it had certain specificity. This study provided experimental materials for increasing the high expression of exogenous gene in bovine muscle cells, and also laid the molecular theoretical basis for obtaining the high specific promoter of bovine muscle and the transgenic beef cattle.
文摘目的评价利妥昔单抗治疗重症肌无力(MG)的有效性和安全性。方法回顾性收集2018年2月至2019年7月北京医院神经内科收治的MG患者21例,所有患者血清抗乙酰胆碱受体(acetylcholine receptor,AChR)抗体或抗肌肉特异性酪氨酸激酶(muscle specific kinase,MuSK)抗体阳性。给予患者4次500mg利妥昔单抗治疗。分析患者治疗前后许氏评分,血CD19阳性B淋巴细胞、CD20阳性B淋巴细胞百分数以及相关抗体变化情况,同时观察利妥昔单抗的不良反应。结果共20例患者在使用4次500mg利妥昔单抗治疗后复查许氏评分,治疗前基线许氏评分为7~44分,平均(18.5±10.56)分,治疗后许氏评分为0~25分,平均(10.45±7.70)分,经利妥昔单抗治疗后患者许氏评分减低为6.5(3.25,12.75)分〔Md(P25,P75)〕,差异有统计学意义(P<0.01)。共16例患者于第1次、第2次、第3次及第4次利妥昔单抗用药后复查许氏评分,与用药前比较许氏评分降低分别为2(0,7)分〔Md(P25,P75)〕以及(6.12±4.54)、(6.18±5.98)、(8.12±5.80)分,与治疗前相比差异均有统计学意义(均P<0.01)。患者外周血CD19、CD20阳性B淋巴细胞计数于第一次用药后分别下降96%、97%。抗AChR抗体阳性患者共19例,用药后不同时间复查抗体水平14次,共发现2例患者抗体滴度降低。共9例患者出现不良反应,均为良性不良反应。结论4次500mg利妥昔单抗治疗MG显示出良好治疗效果,且安全性高,耐受性好。
文摘Muscle Specific Receptor Thyrosine Kinase (MuSK) Myasthenia Gravis (MG) is a rare auto-immune disorder of the neuromuscular junction. The clinical presentation of MG is dominated by fluctuating weakness of the extra-ocular, orofacial and limb muscles. The clinical presentation of MuSK MG can vary, which may delay diagnostic procedures. We present a patient who initially presented with severe weight loss and slowly progressive developing neck extensor weakness and diplopia. A single fiber EMG led to the diagnosis MG and antibodies directed towards MuSK were detected. He was treated with prednisone and the steroid sparing agent azathioprine after which he made full recovery.
