Nucleolar characteristics of reducing bodies in reducing body myopathy.
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.
Shinde
Akiyo
A
Department of Neurology, Kansai Medical University, 570-8507 Moriguchi-city, Japan.
Nakano
Satoshi
S
Kusaka
Hirofumi
H
Nakaya
Yoshifumi
Y
Sawada
Hideyuki
H
Kohara
Nobuo
N
Shibasaki
Hiroshi
H
D000328:Adult; D002466:Cell Nucleolus / Q000473:pathology* / Q000648:ultrastructure; D004576:Electromyography / Q000379:methods; D005260:Female; D034441:Heterogeneous-Nuclear Ribonucleoproteins / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D002479:Inclusion Bodies / Q000473:pathology* / Q000648:ultrastructure; D008854:Microscopy, Electron / Q000379:methods; D009135:Muscular Diseases / Q000378:metabolism / Q000473:pathology*; D009580:Nitroblue Tetrazolium; D013194:Staining and Labeling
D034441:Heterogeneous-Nuclear Ribonucleoproteins; D009580:Nitroblue Tetrazolium