2010 Fiscal Year Final Research Report
Treatment of distal myopathy caused by GNE gene aberration
| Project/Area Number |
20591005
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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 | Oita University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
KIMURA Noriyuki 大分大学, 医学部, 講師 (30433048)
HAZAMA Yusuke 大分大学, 医学部, 助教 (80448547)
OKAZAKI Toshio 大分大学, 医学部, 助教 (00464438)
ARAKAWA Ryuki 大分大学, 医学部, 助教 (90363548)
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| Research Collaborator |
NAKAMURA Kenichiro 大分大学, 医学部, 大学院生
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| Project Period (FY) |
2008 – 2010
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| Keywords | 遠位型ミオパチー / rimmed vacuoles / リソソーム / ユビキチン・プロテアゾーム / GNE / マイクロアレイ / nNOS / IGF-1 |
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| Research Abstract |
GNE was exclusively present in the cytoplasm of mutant GNE gene-transfected cells, which showed normal appearance. Specific 116 genes were detected in GNE-knocked down cells by oligonucleotide microarray. GNE may play a certain role in muscle fiber regeneration.The ubiquitin-proteasome proteolysis pathway as well as lysosomal-proteolysis pathway may mediate the muscle fiber destraction in rimmed vacuolar myopathy such as distal myopathy with rimmed vacuoles and chloroquine-indeced myopathy. IGF-1 treatment may suppress the overdevelopment of rimmed vacuoles in those myopathies.
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[Journal Article] Improvement of deficient natural killer activity and delayed bacterici-dal activity by a thiol proteinase inhibitor, E-64-d, in leukocytes from Chediak-Higashi syndrome patients in vitro2010
Author(s)
Tanabe F, Kasai H, He L, Kin T, Fujikado T, Kumamoto T, Hara T, Iwata T, Ito M
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Journal Title
Int Immunopharmacol 9(3)
Pages: 366-370
Peer Reviewed
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