| First Author | Ruan GX | Year | 2022 |
| Journal | Cell Rep | Volume | 38 |
| Issue | 6 | Pages | 110338 |
| PubMed ID | 35139388 | Mgi Jnum | J:320439 |
| Mgi Id | MGI:6874534 | Doi | 10.1016/j.celrep.2022.110338 |
| Citation | Ruan GX, et al. (2022) The spliceosome component Usp39 controls B cell development by regulating immunoglobulin gene rearrangement. Cell Rep 38(6):110338 |
| abstractText | The spliceosome is a large ribonucleoprotein complex responsible for pre-mRNA splicing and genome stability maintenance. Disruption of the spliceosome activity may lead to developmental disorders and tumorigenesis. However, the physiological role that the spliceosome plays in B cell development and function is still poorly defined. Here, we demonstrate that ubiquitin-specific peptidase 39 (Usp39), a spliceosome component of the U4/U6.U5 tri-snRNP complex, is essential for B cell development. Ablation of Usp39 in B cell lineage blocks pre-pro-B to pro-B cell transition in the bone marrow, leading to a profound reduction of mature B cells in the periphery. We show that Usp39 specifically regulates immunoglobulin gene rearrangement in a spliceosome-dependent manner, which involves modulating chromatin interactions at the Igh locus. Moreover, our results indicate that Usp39 deletion reduces the pre-malignant B cells in Emu-Myc transgenic mice and significantly improves their survival. |
