2010 Fiscal Year Final Research Report
Dynamic O-GlcNAc modification in inclusion body myositis
| Project/Area Number |
20591012
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | Kansai Medical University |
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Principal Investigator |
KUSAKA Hirofumi Kansai Medical University, 医学部, 教授 (70250066)
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| Co-Investigator(Kenkyū-buntansha) |
ITO Hidefumi 関西医科大学, 医学部, 准教授 (20250061)
SHINDE Akiyo 関西医科大学, 医学部, 助教 (10368235)
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| Project Period (FY) |
2008 – 2010
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| Keywords | 脳神経疾患 / 病理学 / ミオパチー / 封入体筋炎 / 核細胞質間輸送 |
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| Research Abstract |
O-linked N-acetylglucosamine (O-GlcNAc) is a ubiquitous posttranslational modification of nucleocytoplasmic proteins, which introduces the attachment of N-acetylglucosamine to serine or threonine residues of a protein. Unlike other protein glycosylation, this modification is highly reversible and like phosphorylation, it plays important roles in various cell signaling. Here, we immunolocalized O-GlcNAc-modified proteins in muscle biopsy specimens. In normal muscle fibers, O-GlcNAc was found along plasma me branes and in nuclei. Diffuse and increased cytoplasmic staining of O-GlcNAc was detected in : 1) regenerating muscle fibers in muscular dystrophy, myositis and rhabdomyolysis ; 2) a proportion of atrophic fibers in myositis, such as those found in perifascicular regions in dermatomyositis ; 3) vacuolated fibers in sporadic inclusion body myositis (s-IBM) and distal myopathy with rimmed vacuoles (DMRV). The increases of O-GlcNAc glycosylation could be associated with the stress response, as these lesions have been shown to be positive for several stress markers. Meanwhile, vacuolar rims in s-IBM and DMRV were sometimes sharply lined by O-GlcNAc-positive deposits, which reflects myonuclear breakdown in the diseases.
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[Journal Article] The first Japanese patient with variant Creutzfeldt-Jakob disease (vCJD).2009
Author(s)
Shinde A, Kunieda T, Kinoshita Y, Wate R, Nakano S, Ito H, Yamada M, Kitamoto T, Nakamura Y, Matsumoto S, Kusaka H
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Journal Title
Neuropathology. 29
Pages: 713-9
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