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Deficiency of ribosomal protein S19 during early embryogenesis leads to reduction of erythrocytes in a zebrafish model of Diamond-Blackfan anemia
http://hdl.handle.net/10458/1905
http://hdl.handle.net/10458/1905aa2f156e-d5d2-487b-b192-5eb8290f68e8
| 名前 / ファイル | ライセンス | アクション |
|---|---|---|
HMG-2008-W-00403.R1-UECHI.pdf (1.5 MB)
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| Item type | 学術雑誌論文 / Journal Article(1) | |||||
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| 公開日 | 2009-03-02 | |||||
| タイトル | ||||||
| タイトル | Deficiency of ribosomal protein S19 during early embryogenesis leads to reduction of erythrocytes in a zebrafish model of Diamond-Blackfan anemia | |||||
| 言語 | en | |||||
| 言語 | ||||||
| 言語 | eng | |||||
| キーワード | ||||||
| 言語 | en | |||||
| 主題Scheme | Other | |||||
| キーワード | nuclear protein, protein rpl35, protein rpl35a, protein rplp2, protein S19, ribosome protein, protein analysis, protein deficiency, protein function, Ribosomal Proteins, Zebrafish Proteins | |||||
| 資源タイプ | ||||||
| 資源タイプ | journal article | |||||
| 著者 |
剣持, 直哉
× 剣持, 直哉× 上地, 珠代× Nakajima, Yukari× Chakraborty, Anirban× Torihara, Hidetsugu× Higa, Sayomi |
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| 抄録 | ||||||
| 内容記述タイプ | Abstract | |||||
| 内容記述 | Ribosomes are responsible for protein synthesis in all cells. Ribosomal protein S19 （RPS19） is one of the 79 ribosomal proteins （RPs） in vertebrates. Heterozygous mutations in RPS19 have been identified in 25% of patients with Diamond-Blackfan anemia （DBA）, but the relationship between RPS19 mutations and the pure red-cell aplasia of DBA is unclear. In this study, we developed an RPS19-deficient zebrafish by knocking down rps19 using a Morpholino antisense oligo. The RPS19-deficient animals showed a dramatic decrease in blood cells as well as deformities in the head and tail regions at early developmental stages. These phenotypes were rescued by injection of zebrafish rps19 mRNA, but not by injection of rps19 mRNAs with mutations that have been identified in DBA patients. Our results indicate that rps19 is essential for hematopoietic differentiation during early embryogenesis. The effects were specific to rps19, but knocking down the genes for three other RPs, rpl35, rpl35a and rplp2, produced similar phenotypes, suggesting that these genes might have a common function in zebrafish erythropoiesis. The RPS19-deficient zebrafish will provide a valuable tool for investigating the molecular mechanisms of DBA development in humans. | |||||
| 言語 | en | |||||
| 書誌情報 |
en : Human molecular genetics 巻 17, 号 20, p. 3204-3211, 発行日 2008-07 |
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| 出版者 | ||||||
| 出版者 | Oxford University Press | |||||
| 言語 | en | |||||
| ISSN | ||||||
| 収録物識別子タイプ | ISSN | |||||
| 収録物識別子 | 09646906 | |||||
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| 収録物識別子タイプ | NCID | |||||
| 収録物識別子 | AA10836720 | |||||
| 著者版フラグ | ||||||
| 出版タイプ | AM | |||||
