1999年
Polyglutamine domain proteins with expanded repeats bind neurotilament, altering the neurofilament network
OXIDATIVE/ENERGY METABOLISM IN NEURODEGENERATIVE DISORDERS
- ,
- ,
- ,
- 巻
- 893
- 号
- 開始ページ
- 192
- 終了ページ
- 202
- 記述言語
- 英語
- 掲載種別
- 研究論文(学術雑誌)
- DOI
- 10.1111/j.1749-6632.1999.tb07826.x
- 出版者・発行元
- NEW YORK ACAD SCIENCES
Proteins with expanded polyglutamine (polyQ) repeats cause eight inherited neurodegenerative diseases, Nuclear and cytoplasmic polyQ protein is a common feature of these diseases, but its role in cell death remains debatable. Since the neuronal intermediate filament network is composed of neurofilament (NF) and NF abnormalities occur in neurodegenerative diseases, we examined whether pathologic-length polyQ domain proteins interact with NF. me expressed polyQ-green fluorescent fusion proteins (GFP) in a neuroblast cell line, TR1. Pathologic-length polyQ-GFP fusion proteins form large cytoplasmic aggregates surrounded by neurofilament, Immunoisolation of pathologic-length polyQ proteins co-isolated 68 kD NF protein demonstrating molecular interaction. These observations suggest that polyQ interaction with NF is important in the pathogenesis of the polyglutamine repeat disease.
- リンク情報
- ID情報
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- DOI : 10.1111/j.1749-6632.1999.tb07826.x
- ISSN : 0077-8923
- PubMed ID : 10672238
- Web of Science ID : WOS:000085330600015