Department of Biomedical Sciences, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, Serdang, Selangor, Malaysia.
Department of Human Anatomy, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, 43400, Serdang, Selangor, Malaysia.
Biochem Biophys Res Commun. 2024 Nov 26;735:150664. doi: 10.1016/j.bbrc.2024.150664. Epub 2024 Sep 4.
In individuals with Down syndrome (DS), an additional HSA21 chromosome copy leads to the overexpression of a myriad of HSA21 genes, disrupting the transcription of the entire genome. This dysregulation in transcription and post-transcriptional modifications contributes to abnormal phenotypes across nearly all tissues and organs in DS individuals. The array of severe clinical symptoms associated with trisomy 21 poses a considerable challenge in the quest for a cure for DS. Fortunately, a wealth of research suggests that chromosome therapy, hinging on cutting-edge genome editing technologies, can potentially eliminate the extra copy of the human chromosome 21. Genome editing tools have demonstrated their efficacy in restoring trisomy to a normal diploid state in vitro DS cell models. Furthermore, we delve into the noteworthy findings in cellular therapy for DS, with recent studies showcasing the increasing feasibility of strategies involving stem cells and CAR T-cells to address corresponding clinical phenotypes.
在唐氏综合征(DS)患者中,第 21 号染色体的额外拷贝导致大量 HSA21 基因的过度表达,扰乱了整个基因组的转录。这种转录和转录后修饰的失调导致 DS 个体几乎所有组织和器官的异常表型。与 21 三体相关的一系列严重临床症状给寻找 DS 的治疗方法带来了巨大的挑战。幸运的是,大量研究表明,基于先进基因组编辑技术的染色体治疗可能有潜力消除人类 21 号染色体的额外拷贝。基因组编辑工具已证明其在体外 DS 细胞模型中将三体恢复为正常二倍体状态的功效。此外,我们深入探讨了 DS 细胞治疗的显著发现,最近的研究表明,涉及干细胞和 CAR T 细胞的策略越来越可行,以解决相应的临床表型。