上皮剪接调节蛋白 1 和生长激素腺瘤中的选择性剪接。
Epithelial splicing regulator protein 1 and alternative splicing in somatotroph adenomas.
机构信息
Section of Specialized Endocrinology and Research Institute for Internal Medicine, Oslo University Hospital, Rikshospitalet, University of Oslo, PO Box 4950 Nydalen, 0424 Oslo, Norway.
出版信息
Endocrinology. 2013 Sep;154(9):3331-43. doi: 10.1210/en.2013-1051. Epub 2013 Jul 3.
Somatotroph adenomas secrete supraphysiological amounts of GH, causing acromegaly. We have previously hypothesized that epithelial mesenchymal transition (EMT) may play a central role in the progression of these adenomas and that epithelial splicing regulator 1 (ESRP1) may function prominently as a master regulator of the EMT process in pituitary adenomas causing acromegaly. To further elucidate the role of ESRP1 in somatotroph adenomas and in EMT progression, we used RNA sequencing (RNAseq) to sequence somatotroph adenomas characterized by high and low ESRP1 levels. Transcripts identified by RNAseq were analyzed in 65 somatotroph adenomas and in GH-producing pituitary rat cells with a specific knockdown of Esrp1. The clinical importance of the transcripts was further investigated by correlating mRNA expression levels with clinical indices of disease activity and treatment response. Many of the transcripts and isoforms identified by RNAseq and verified by quantitative PCR were involved in vesicle transport and calcium signaling and were associated with clinical outcomes. Silencing Esrp1 in GH3 cells resulted in changes of gene expression overlapping the data observed in human somatotroph adenomas and revealed a decreased granulation pattern and attenuated GH release. We observed an alternative splicing pattern for F-box and leucine-rich repeat protein 20, depending on the ESPR1 levels and on changes in circulating IGF-I levels after somatostatin analog treatment. Our study indicates that ESRP1 in somatotroph adenomas regulates transcripts that may be essential in the EMT progression and in the response to somatostatin analog treatment.
生长激素腺瘤分泌超生理量的 GH,导致肢端肥大症。我们之前假设上皮间质转化(EMT)可能在这些腺瘤的进展中起核心作用,并且上皮剪接调节因子 1(ESRP1)可能作为导致肢端肥大症的垂体腺瘤 EMT 过程中的主要调节因子发挥重要作用。为了进一步阐明 ESRP1 在生长激素腺瘤和 EMT 进展中的作用,我们使用 RNA 测序(RNAseq)对 ESRP1 水平高低不同的生长激素腺瘤进行测序。通过 RNAseq 鉴定的转录本在 65 例生长激素腺瘤和 GH 分泌性垂体大鼠细胞中进行了分析,这些细胞中 Esrp1 被特异性敲低。通过将 mRNA 表达水平与疾病活动和治疗反应的临床指标相关联,进一步研究了转录本的临床重要性。通过 RNAseq 鉴定并通过定量 PCR 验证的许多转录本和异构体参与囊泡运输和钙信号转导,并与临床结果相关。在 GH3 细胞中沉默 Esrp1 导致基因表达的变化与在人类生长激素腺瘤中观察到的数据重叠,并显示出颗粒减少的模式和减弱的 GH 释放。我们观察到 F-box 和富含亮氨酸重复蛋白 20 的可变剪接模式,这取决于 ESPR1 水平以及生长抑素类似物治疗后循环 IGF-I 水平的变化。我们的研究表明,生长激素腺瘤中的 ESRP1 调节可能在 EMT 进展和生长抑素类似物治疗反应中起重要作用的转录本。