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- KUMAMOTO Toshihide
- The Third Department of Internal Medicine, Oita Medical University
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- FUJIMOTO Shin
- The Third Department of Internal Medicine, Oita Medical University
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- NAGAO Shin-ichiro
- The Third Department of Internal Medicine, Oita Medical University
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- MASUDA Tomoko
- The Third Department of Internal Medicine, Oita Medical University
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- SUGIHARA Rie
- The Third Department of Internal Medicine, Oita Medical University
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- UEYAMA Hidetsugu
- The Third Department of Internal Medicine, Oita Medical University
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- TSUDA Tomiyasu
- The Third Department of Internal Medicine, Oita Medical University
書誌事項
- タイトル別名
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- Proteasomes in Distal Myopathy with Rim
この論文をさがす
抄録
In a previous report we suggested that muscle fibers in distal myopathy with rimmed vacuoles (DMRV) were degraded by both lysosomal proteolysis (cathepsins) and Ca2+-dependent, nonlysosomal proteolysis (ealpain). Given recent evidence of abnormal ubiquitin accumulation in rimmed vacuoles, we examined the role of the ATP-ubiquitin-dependent proteolytic pathway (proteasomes) in myofiber degradation in this myopathy. Immunohistochemically, proteasomes (26S) were located in the cytoplasm in normal human muscle, but the staining intensity was weak. Quantitative analysis showed more reactivity for proteasomes in DMRV muscles and, to a lesser extent, in muscles from muscular dystrophy, polymyositis, and amyotrophic lateral sclerosis patients. In DMRV, proteasomes often were located within or on the rim of rimmed vacuoles, and in the cytoplasm of atrophic fibers. Ubiquitin accumulation was marked within rimmed vacuoles and was seen less extensively in the cytoplasm of atrophic fibers. The latter proteins colocalized well. In other diseased muscles, proteasomes and ubiquitin showed a positive reaction in the atrophic or necrotic fibers. The results indicate increased proteasome and ubiquitin in these muscle fibers as well as in other diseased muscle fibers. We suggest that the ATP-ubiquitin-proteasome proteolytic pathway as well as the nonlysosomal ealpain and the lysosomal proteolytic pathway may participate in the muscle fiber degradation in DMRV. <br>(Internal Medicine 37: 746-752, 1998)
収録刊行物
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- Internal Medicine
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Internal Medicine 37 (9), 746-752, 1998
一般社団法人 日本内科学会
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詳細情報 詳細情報について
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- CRID
- 1390001204869350528
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- NII論文ID
- 10007605372
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- NII書誌ID
- AA10827774
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- COI
- 1:STN:280:DyaK1M%2FhvFahtA%3D%3D
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- ISSN
- 13497235
- 09182918
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- NDL書誌ID
- 4579220
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- PubMed
- 9804081
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- 本文言語コード
- en
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- データソース種別
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- JaLC
- NDL
- Crossref
- PubMed
- CiNii Articles
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- 抄録ライセンスフラグ
- 使用不可