Document Detail

Optineurin is potentially associated with TDP-43 and involved in the pathogenesis of inclusion body myositis.
MedLine Citation:
PMID:  22860700     Owner:  NLM     Status:  Publisher    
Aims: Increasing evidences suggest a similarity in the pathophysiological mechanisms of neuronal cell death in amyotrophic lateral sclerosis (ALS) and myofibre degeneration in sporadic inclusion body myositis (sIBM). The aim of this study is to elucidate the involvement of ALS-causing proteins in the pathophysiological mechanisms in sIBM. Methods: Skeletal muscle biopsy specimens of 5 patients with sIBM, 2 with oculopharyngeal muscular dystrophy (OPMD), 3 with polymyositis (PM), 3 with dermatomyositis (DM), 3 with neurogenic muscular atrophy, and 3 healthy control subjects were examined. We analyzed the expression and localization of familial ALS-causing proteins, including transactive response DNA binding protein-43 (TDP-43), fused in sarcoma/translocated in liposarcoma (FUS/TLS), Cu/Zn superoxide dismutase (SOD1), and optineurin (OPTN) by immunohistochemistry. Results: TDP-43, OPTN, and to a lesser extent FUS/TLS were more frequently accumulated in the cytoplasm in patients with sIBM and OPMD than in patients with PM, DM, neurogenic muscular atrophy, or healthy control subjects. SOD1 was accumulated in a small percentage of myofibres in patients with sIBM and OPMD, and to a very small extent in patients with PM and DM. Confocal microscopy imaging showed that TDP-43 proteins more often colocalized with OPTN than with FUS/TLS, p62, and phosphorylated Tau. Conclusions: These findings suggest that OPTN in cooperation with TDP-43 might be involved in the pathophysiological mechanisms of skeletal muscular degeneration in myopathy with rimmed vacuoles. Further investigation into these mechanisms is therefore warranted. © 2012 The Authors. Neuropathology and Applied Neurobiology © 2012 British Neuropathological Society.
Satoshi Yamashita; En Kimura; Nozomu Tawara; Hideya Sakaguchi; Tatsuya Nakama; Yasushi Maeda; Teruyuki Hirano; Makoto Uchino; Yukio Ando
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-8-3
Journal Detail:
Title:  Neuropathology and applied neurobiology     Volume:  -     ISSN:  1365-2990     ISO Abbreviation:  Neuropathol. Appl. Neurobiol.     Publication Date:  2012 Aug 
Date Detail:
Created Date:  2012-8-6     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7609829     Medline TA:  Neuropathol Appl Neurobiol     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
© 2012 The Authors. Neuropathology and Applied Neurobiology © 2012 British Neuropathological Society.
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan.
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