論文 - 詳細
| RRC ID | 61672 |
|---|---|
| 著者 | Arai Y, Takami M, An Y, Matsuo-Takasaki M, Hemmi Y, Wakabayashi T, Inoue J, Noguchi M, Nakamura Y, Sugimoto K, Takemura T, Okita K, Osafune K, Takasato M, Hayata T, Hayashi Y. |
| タイトル | Generation of two human induced pluripotent stem cell lines derived from two juvenile nephronophthisis patients with NPHP1 deletion. |
| ジャーナル | Stem Cell Res |
| Abstract |
Juvenile nephronophthisis is an inherited renal ciliopathy, causing cystic kidney disease, renal fibrosis, and end-stage renal failure. Human induced pluripotent stem cell (hiPSC) lines, derived from two Juvenile nephronophthisis patients, were generated from peripheral blood mononuclear cells by episomal plasmid vectors. Generated hiPSC lines showed self-renewal and pluripotency and carried a large deletion in NPHP1 (Nephrocystin 1) gene. Since the molecular pathogenesis caused by NPHP1 dysfunction remains unclear, these cell resources provide useful tools to establish disease models and to develop new therapies for juvenile nephronophthisis. |
| 巻・号 | 45 |
| ページ | 101815 |
| 公開日 | 2020-5-1 |
| DOI | 10.1016/j.scr.2020.101815 |
| PII | S1873-5061(20)30117-3 |
| PMID | 32361464 |
| MeSH | Adaptor Proteins, Signal Transducing Cytoskeletal Proteins Fibrosis Humans Induced Pluripotent Stem Cells* Kidney Diseases, Cystic / congenital Leukocytes, Mononuclear Membrane Proteins / genetics |
| IF | 4.495 |
| オルトメトリクス指標 |
オルトメトリクス指標項目
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| 最多言及媒体 | News |
| 各媒体での言及数の合計 | 4 |
| 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 | |
| ヒト・動物細胞 | HPS0447 HPS0450 Vero(RCB0001) |