Abstract:
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 five patients with sIBM, two with oculopharyngeal muscular dystrophy (OPMD), three with polymyositis (PM), three with dermatomyositis (DM), three with neurogenic muscular atrophy, and three healthy control subjects were examined. We analysed 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.
journal_name
Neuropathol Appl Neurobioljournal_title
Neuropathology and applied neurobiologyauthors
Yamashita S,Kimura E,Tawara N,Sakaguchi H,Nakama T,Maeda Y,Hirano T,Uchino M,Ando Ydoi
10.1111/j.1365-2990.2012.01297.xsubject
Has Abstractpub_date
2013-06-01 00:00:00pages
406-16issue
4eissn
0305-1846issn
1365-2990journal_volume
39pub_type
杂志文章abstract:AIMS:Plectin, a universally expressed multi-functional cytolinker protein, is crucial for intermediate filament networking, including crosstalk with actomyosin and microtubules. In addition to its involvement in a number of diseases affecting skin, skeletal muscle, heart, and other stress-exposed tissues, indications f...
journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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doi:
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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doi:10.1111/j.1365-2990.1995.tb01066.x
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abstract::Multiple system atrophy (MSA) is an unrelenting, sporadic, adult-onset, neurodegenerative disease of unknown aetiology. Its clinically progressive course is characterized by a variable combination of parkinsonism, cerebellar ataxia and/or autonomic dysfunction. Neuropathological examination often reveals gross abnorma...
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pub_type: 杂志文章,评审
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1978.tb00548.x
更新日期:1978-07-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
pub_type: 杂志文章
doi:10.1111/j.1365-2990.1988.tb00864.x
更新日期:1988-01-01 00:00:00
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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doi:10.1111/j.1365-2990.1991.tb00692.x
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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journal_title:Neuropathology and applied neurobiology
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