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Nucleolar characteristics of reducing bodies in reducing body myopathy

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Abstract

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.

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Acknowledgements

This work was supported in part by a Grant-in-Aid for Scientific Research from JSPS (C-13670676) and a Grant from Kansai Medical University (Research Grant B). We thank Ms. Hitomi Nakabayashi for technical assistance.

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Authors and Affiliations

  1. Department of Neurology, Kansai Medical University, 570-8507, Moriguchi-city, Japan

    Akiyo Shinde, Satoshi Nakano & Hirofumi Kusaka

  2. Department of Neurology, Graduate School of Medicine, Kyoto University, Kyoto, Japan

    Akiyo Shinde, Yoshifumi Nakaya, Hideyuki Sawada, Nobuo Kohara & Hiroshi Shibasaki

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  1. Akiyo Shinde
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  2. Satoshi Nakano
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  3. Hirofumi Kusaka
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  4. Yoshifumi Nakaya
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  5. Hideyuki Sawada
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  6. Nobuo Kohara
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  7. Hiroshi Shibasaki
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Correspondence to Satoshi Nakano.

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Shinde, A., Nakano, S., Kusaka, H. et al. Nucleolar characteristics of reducing bodies in reducing body myopathy. Acta Neuropathol 107, 265–271 (2004). https://doi.org/10.1007/s00401-003-0806-y

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  • DOI: https://doi.org/10.1007/s00401-003-0806-y

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