論文 - 詳細
| RRC ID | 286 |
|---|---|
| 著者 | Takeyama K, Ito S, Yamamoto A, Tanimoto H, Furutani T, Kanuka H, Miura M, Tabata T, Kato S. |
| タイトル | Androgen-dependent neurodegeneration by polyglutamine-expanded human androgen receptor in Drosophila. |
| ジャーナル | Neuron |
| Abstract |
Spinal and bulbar muscular atrophy (SBMA) is an X-linked, adult-onset, neurodegenerative disorder affecting only males and is caused by expanded polyglutamine (polyQ) stretches in the N-terminal A/B domain of human androgen receptor (hAR). Although no overt phenotype was detected in adult fly eye photoreceptor neurons expressing mutant hAR (polyQ 52), ingestion of androgen or its known antagonists caused marked neurodegeneration with nuclear localization and structural alteration of the hAR mutant. Ligand-independent toxicity was detected with a truncated polyQ-expanded A/B domain alone, which was attenuated with cytosolic trapping by coexpression of the unliganded hAR E/F ligand binding domain. Thus, our findings suggest that the full binding of androgen to the polyQ-expanded hAR mutants leads to structural alteration with nuclear translocation that eventually results in the onset of SBMA in male patients. |
| 巻・号 | 35(5) |
| ページ | 855-64 |
| 公開日 | 2002-8-29 |
| DOI | 10.1016/s0896-6273(02)00875-9 |
| PII | S0896627302008759 |
| PMID | 12372281 |
| MeSH | Animals Animals, Genetically Modified / genetics Binding Sites / genetics COS Cells Drosophila / genetics* Gene Targeting Humans Muscular Atrophy, Spinal / genetics Muscular Atrophy, Spinal / metabolism Neurodegenerative Diseases / genetics Neurodegenerative Diseases / metabolism* Peptides / metabolism* Photoreceptor Cells, Invertebrate / metabolism Receptors, Androgen / genetics Receptors, Androgen / metabolism Receptors, Androgen / physiology* |
| IF | 14.415 |
| 引用数 | 224 |
| WOS 分野 | NEUROSCIENCES |
| オルトメトリクス指標 |
オルトメトリクス指標項目
|
| 最多言及媒体 | X(Twitter) |
| 各媒体での言及数の合計 | 2 |
| 過去6か月間でのオルトメトリクス指標の変動値 | 0.0 |
| リソース情報 | |
| ショウジョウバエ | |