小基因
Jurkat细胞
外显子
生物
RNA剪接
T细胞受体
癌症研究
选择性拼接
细胞生物学
FOXP3型
ZAP70型
信号转导
免疫学
外显子剪接增强剂
分子生物学
拼接因子
小干扰RNA
免疫系统
免疫耐受
外显子跳跃
T细胞
CD3型
异位表达
和平号-155
调节性T细胞
免疫沉淀
作者
Xiang Hu,Shaoqiu Leng,Yan Liu,Ruxia Zhao,Xinyue Liu,Ju Li,Cheng Zhang,Caiyun Meng,Haoran Liu,Yanqi Zhang,Chaoyang Li,Yuxin Wang,Qi Feng,Nan Jiang,Shuqian Xu,Shuwen Wang,Jun Peng
出处
期刊:Blood
[Elsevier BV]
日期:2026-08-17
标识
DOI:10.1182/blood.2026033543
摘要
Immune thrombocytopenia (ITP) is an autoimmune disorder characterized by antibody-mediated platelet destruction and impaired regulatory T-cell (Treg) function, yet its molecular basis remains poorly defined. Here, we show that CD4+ naive T cells from patients with ITP exhibit diminished T-cell receptor (TCR) signaling and impaired in vitro Treg induction relative to healthy controls. RNA-sequencing revealed widespread alternative splicing dysregulation, most notably exon 8 skipping in LCK, a kinase central to TCR signaling and Treg differentiation. Using an LCK minigene combined with RNA pull-down mass spectrometry and RNA immunoprecipitation assays, we identified the splicing factor SRSF1 as a direct upstream regulator of LCK exon 8 skipping. Notably, SRSF1 expression was reduced in CD4+ naive T cells from ITP patients, and its overexpression restored in vitro Treg induction capacity. Antisense oligonucleotide (ASO)-mediated blockade of SRSF1 binding to LCK enhanced exon 8 skipping and attenuated TCR activation in Jurkat cells. Although murine Lck lacks the human-specific recursive splicing sites required for exon 8 exclusion, adoptive transfer of CD4+ naive T cells expressing the exon 8-skipped murine Lck into CD61-knockout mice significantly reduced Treg proportions and platelet counts in an active ITP model. Mechanistically, Jurkat cells engineered to express only the exon 8-skipped LCK variant showed markedly reduced binding to ZAP70 and CD3ζ, which may partly account for the attenuated TCR signaling and downstream FOXP3 induction. Together, these findings define a novel SRSF1-LCK splicing axis that may regulate Treg development in ITP.
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