Nucleolar characteristics of reducing bodies in reducing body myopathy
Nucleolar characteristics of reducing bodies in reducing body myopathy
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DOI：
10.1007/s00401-003-0806-y
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发表时间：
2004-01
影响因子：
12.7
通讯作者：
A. Shinde;S. Nakano;H. Kusaka;Yoshifumi Nakaya;H. Sawada;N. Kohara;H. Shibasaki
中科院分区：
文献类型：
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作者：
A. Shinde;S. Nakano;H. Kusaka;Yoshifumi Nakaya;H. Sawada;N. Kohara;H. Shibasaki
还原体肌病是一种罕见的肌肉疾病，其定义为受影响的肌纤维中的异常内含物，可以用甲萘醌-硝基蓝四唑染色。这些夹杂物的来源尚未确定。在这里，我们表明，还原体具有核仁的特征。超微结构，肌肉活检标本与成人发病的还原体肌病患者显示颗粒结构的还原体与功能相似的颗粒的核仁，其中包括前核糖体。此外，还原体的嗜银核仁组织者区的组织化学（用于检测核糖体RNA在核仁中活跃转录的区域的方法），以及针对核仁和核核糖核蛋白的抗体呈阳性。目前的研究结果表明，还原体含有前核糖体及其相关蛋白质的核仁和还原体的形成可能是由于缺陷的加工和组装的核糖体。
Reducing body myopathy is a rare muscle disease defined by abnormal inclusions in affected muscle fibers that can be stained with menadione-nitroblue tetrazolium. The origin of these inclusions has not been determined. Here we show that reducing bodies bear characteristics of nucleoli. Ultrastructurally, muscle biopsy specimens of a patient with adult-onset reducing body myopathy showed granular structures of reducing bodies with features similar to the granules of nucleoli, which consisted of pre-ribosomes. In addition, reducing bodies were positive for histochemistry of argyrophilic nucleolar organizer regions (a method for detecting the areas where ribosomal RNA is actively transcribed in the nucleolus), and for antibodies against nucleoli and nuclear ribonucleoprotein. The current findings suggest that reducing bodies contain pre-ribosomes and their associated proteins of the nucleolus and that formation of reducing bodies may result from defects of processing and assembly of ribosomes.