9GMO の概要
| エントリーDOI | 10.2210/pdb9gmo/pdb |
| EMDBエントリー | 51452 |
| 分子名称 | 28S rRNA, 60S ribosomal protein L17, 60S ribosomal protein L18, ... (53 entities in total) |
| 機能のキーワード | complex, pre-60s, ul16, ribosome |
| 由来する生物種 | Homo sapiens (human) 詳細 |
| タンパク質・核酸の鎖数 | 46 |
| 化学式量合計 | 2645876.12 |
| 構造登録者 | |
| 主引用文献 | Akers, J.,Bothe, A.,Suh, H.,Jung, C.,Stolp, Z.,Ghosh, T.,Yan, L.,Wang, Y.,Grismer, T.,Reyes, A.,Hu, T.,Xu, S.,Ban, N.,Kostova, K. ZNF574 is a Quality Control Factor For Defective Ribosome Biogenesis Intermediates. Biorxiv, 2024 Cited by PubMed Abstract: Eukaryotic ribosome assembly is an intricate process that involves four ribosomal RNAs, 80 ribosomal proteins, and over 200 biogenesis factors that take part in numerous interdependent steps. This complexity creates a large genetic space in which pathogenic mutations can occur. Dead-end ribosome intermediates that result from biogenesis errors are rapidly degraded, affirming the existence of quality control pathway(s) that monitor ribosome assembly. However, the factors that differentiate between on-path and dead-end intermediates are unknown. We engineered a system to perturb ribosome assembly in human cells and discovered that faulty ribosomes are degraded via the ubiquitin proteasome system. We identified ZNF574 as a key component of a novel quality control pathway, which we term the Ribosome Assembly Surveillance Pathway (RASP). Loss of ZNF574 results in the accumulation of faulty biogenesis intermediates that interfere with global ribosome production, further emphasizing the role of RASP in protein homeostasis and cellular health. PubMed: 38746087DOI: 10.1101/2024.04.26.591394 主引用文献が同じPDBエントリー |
| 実験手法 | ELECTRON MICROSCOPY (2.59 Å) |
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