Heydemann Ahlke, Siemionow Maria
Department of Physiology and Biophysics, University of Illinois at Chicago, Chicago, IL 60607, USA.
Center for Cardiovascular Research, University of Illinois at Chicago, Chicago, IL 60607, USA.
Biomedicines. 2023 Mar 9;11(3):830. doi: 10.3390/biomedicines11030830.
Despite the full cloning of the Dystrophin cDNA 35 years ago, no effective treatment exists for the Duchenne Muscular Dystrophy (DMD) patients who have a mutation in this gene. Many treatment options have been considered, investigated preclinically and some clinically, but none have circumvented all barriers and effectively treated the disease without burdening the patients with severe side-effects. However, currently, many novel therapies are in the pipelines of research labs and pharmaceutical companies and many of these have progressed to clinical trials. A brief review of these promising therapies is presented, followed by a description of two novel technologies that when utilized together effectively treat the disease in the mdx mouse model. One novel technology is to generate chimeric cells from the patient's own cells and a normal donor. The other technology is to systemically transplant these cells into the femur via the intraosseous route.
尽管35年前就已完成肌营养不良蛋白cDNA的全克隆,但对于该基因发生突变的杜氏肌营养不良症(DMD)患者,目前尚无有效的治疗方法。人们已经考虑了许多治疗方案,并在临床前和一些临床研究中进行了调查,但没有一种方案能够克服所有障碍并有效治疗该疾病,同时又不给患者带来严重的副作用。然而,目前许多新型疗法正处于研究实验室和制药公司的研发进程中,其中许多已经进入临床试验阶段。本文简要回顾了这些有前景的疗法,随后描述了两种新技术,将它们联合使用可有效治疗mdx小鼠模型中的疾病。一种新技术是从患者自身细胞和正常供体细胞中生成嵌合细胞。另一种技术是通过骨内途径将这些细胞系统性地移植到股骨中。