Biological significance of the expression of a Wilson disease mutant protein
| Project/Area Number |
14570528
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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 |
Gastroenterology
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| Research Institution | Kurume University |
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Principal Investigator |
HARADA Masaru Kurume University, Assistant Professor, 医学部, 講師 (00241175)
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| Co-Investigator(Kenkyū-buntansha) |
TANIMURA Takuji Kurume University, Associate Professor, 医学部, 助教授 (60197986)
KOGA Hironori Kurume University, Assistant Professor, 医学部, 講師 (90268855)
NAKAMURA Toru Kurume University, Post doctoral fellow, 医学部, 助手 (30341332)
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| Project Period (FY) |
2002 – 2004
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| Project Status |
Completed (Fiscal Year 2004)
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| Budget Amount *help |
¥3,900,000 (Direct Cost: ¥3,900,000)
Fiscal Year 2004: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 2003: ¥1,400,000 (Direct Cost: ¥1,400,000)
Fiscal Year 2002: ¥1,600,000 (Direct Cost: ¥1,600,000)
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| Keywords | Wilson disease / Cooper / ATP7B / Mallory body / Inclusion body / 中間系線維 / マロリー体 / aggresome / プロテアソームゴルジ装置 / 中間径フィラメント / ユビキチン / プロテアソーム |
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| Research Abstract |
We have examined the intracellular localization of Wilson disease protein ATP7B. While we described the late endosomal localization of ATP7B, others have been reported the different results. In the present study we confirmed that this protein resides in the late endosomes using various cell lines and expression vectors for the late endosome specific proteins (Int J Mol Med 11;293-298:2003, Am J Pathol 166;499-510:2005).
Previously we reported that expression of a Wilson disease mutant protein (ATP7B H1069Q) induced formation of intracellular inclusion bodies similar to Mallory body. We demonstrated inhibition of proteasome function induced similar inclusion bodies and the formation of the inclusion body affected the architecture of the Golgi apparatus. Therefore, formation of the inclusion body may affect the function of the Golgi apparatus (Exp Cell Res 288;6069:2003, Cell Motil Cytoskel 57;37-52:2004). Furthermore, the inclusion body induced by proteasome inhibition reversely disappeared after the removal of the inhibitor. This process was associated with the autophagic degradation (Exp Cell Res 288;60-69:2003). Furthermore, the formation of the inclusion body affected the distribution of cytosolic proteins and might affect the function of these proteins (Exp Cell Res in press).
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Report
(4 results)
Research Products
(35 results)
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[Book] 肝疾患Review2004
Author(s)
原田 大
Total Pages
237
Publisher
日本メデイカルセンター
Description
「研究成果報告書概要(和文)」より
Related Report
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[Book] 肝疾患と生体元素2003
Author(s)
原田 大 他
Total Pages
214
Publisher
日本学会事務センター
Description
「研究成果報告書概要(和文)」より
Related Report
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[Book] 病気の形態学2002
Author(s)
原田 大 他
Total Pages
265
Publisher
学際企画
Description
「研究成果報告書概要(和文)」より
Related Report
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