Moon Jangsup
Rare Disease Center, Department of Genomic Medicine; Department of Neurology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Encephalitis. 2025 Apr;5(2):27-35. doi: 10.47936/encephalitis.2024.00122. Epub 2024 Dec 10.
Tandem repeat disorders (TRDs) are genetic conditions characterized by the abnormal expansion of repetitive DNA sequences within specific genes. The growing number of identified TRDs highlights their complexity, with varied molecular mechanisms ranging from toxic protein production and repeat-associated non-AUG translation to RNA toxicity and epigenetic modifications. TRDs also exhibit unique clinical features such as reduced penetrance, anticipation, and repeat motif changes. Advances in molecular diagnostics such as long-read sequencing have significantly improved the detection of TRDs, especially for large or complex repeat expansions. Additionally, emerging therapeutic strategies, particularly antisense oligonucleotides (ASOs) and gene editing technologies, are showing great promise. ASOs in particular have demonstrated success through mechanisms like allele-specific knockdown and splice modulation. In this review, we explore the classification of TRDs, advances in diagnostics, molecular mechanisms, clinical features, and innovative therapeutic strategies, highlighting the need for further research to refine treatments and improve outcomes.
串联重复序列疾病(TRDs)是一类遗传性疾病,其特征是特定基因内的重复性DNA序列异常扩增。已鉴定出的TRDs数量不断增加,凸显了它们的复杂性,其分子机制多种多样,从有毒蛋白质产生、重复序列相关的非AUG翻译到RNA毒性和表观遗传修饰不等。TRDs还表现出独特的临床特征,如外显率降低、遗传早现和重复基序变化。长读长测序等分子诊断技术的进步显著提高了TRDs的检测水平,尤其是对于大型或复杂的重复序列扩增。此外,新兴的治疗策略,特别是反义寡核苷酸(ASOs)和基因编辑技术,显示出巨大的潜力。特别是ASOs已通过等位基因特异性敲低和剪接调节等机制取得了成功。在这篇综述中,我们探讨了TRDs的分类、诊断进展、分子机制、临床特征和创新治疗策略,强调了进一步研究以优化治疗方法和改善治疗效果的必要性。