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首页> 外文期刊>European Journal of Histochemistry >Cultured myoblasts from patients affected by myotonic dystrophy type 2 exhibit senescence-related features: ultrastructural evidence
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Cultured myoblasts from patients affected by myotonic dystrophy type 2 exhibit senescence-related features: ultrastructural evidence

机译：受2型强直性营养不良患者影响的培养成肌细胞具有衰老相关特征：超微结构证据

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摘要

Myotonic dystrophy type 2 (DM2) is an autosomal dominant disorder caused by the expansion of the tetranucleotidic repeat (CCTG)n in the first intron of the Zinc Finger Protein-9 gene. In DM2 tissues, the expanded mutant transcripts accumulate in nuclear focal aggregates where splicing factors are sequestered, thus affecting mRNA processing. Interestingly, the ultrastructural alterations in the splicing machinery observed in the myonuclei of DM2 skeletal muscles are reminiscent of the nuclear changes occurring in age-related muscle atrophy. Here, we investigated in vitro structural and functional features of satellite cell-derived myoblasts from biceps brachii, in the attempt to investigate cell senescence indices in DM2 patients by ultrastructural cytochemistry. We observed that in satellite cell-derived DM2 myoblasts, cell-senescence alterations such as cytoplasmic vacuolization, reduction of the proteosynthetic apparatus, accumulation of heterochromatin and impairment of the pre-mRNA maturation pathways occur earlier than in myoblasts from healthy patients. These results, together with preliminary in vitro observations on the early onset of defective structural features in DM2 myoblast derived-myotubes, suggest that the regeneration capability of DM2 satellite cells may be impaired, thus contributing to the muscular dystrophy in DM2 patients.
机译：2型肌强直性营养不良（DM2）是一种常染色体显性遗传疾病，由Zinc Finger Protein-9基因的第一个内含子中四核苷酸重复序列（CCTG）n的扩增引起。在DM2组织中，扩展的突变体转录物积聚在核焦点聚集体中，在那里剪接因子被隔离，从而影响mRNA的加工。有趣的是，在DM2骨骼肌的肌核中观察到的剪接机制的超微结构改变使人联想到与年龄相关的肌肉萎缩中发生的核变化。在这里，我们调查了肱二头肌从卫星细胞衍生的成肌细胞的体外结构和功能特征，以试图通过超微结构细胞化学研究DM2患者的细胞衰老指数。我们观察到，在卫星细胞衍生的DM2成肌细胞中，细胞衰老改变（如细胞质空泡化，蛋白质合成设备的减少，异染色质的积累和mRNA前成熟途径的损伤）比健康患者的成肌细胞更早发生。这些结果，加上对DM2成肌细胞衍生的肌管中早期出现的结构特征缺陷的初步体外观察，表明DM2卫星细胞的再生能力可能受损，从而导致DM2患者的肌营养不良。

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