University of Arkansas for Medical Sciences, Little Rock, AR, USA.
Celgene Institute for Translational Research Europe, Sevilla, Spain.
Haematologica. 2021 Mar 1;106(3):736-745. doi: 10.3324/haematol.2019.235424.
Disruption of the normal splicing patterns of RNA is a major factor in the pathogenesis of a number of diseases. Increasingly research has shown the strong influence that splicing patterns can have on cancer progression. Multiple Myeloma is a molecularly heterogeneous disease classified by the presence of key translocations, gene expression profiles and mutations but the splicing patterns in MM remains largely unexplored. We take a multifaceted approach to define the extent and impact of alternative splicing in MM. We look at the spliceosome component, SF3B1, with hotspot mutations (K700E and K666T/Q) shown to result in an increase in alternative splicing in other cancers. We discovered a number of differentially spliced genes in comparison of the SF3B1 mutant and wild type samples that included, MZB1, DYNLL1, TMEM14C and splicing related genes DHX9, CLASRP, and SNRPE. We identified a broader role for abnormal splicing showing clear differences in the extent of novel splice variants in the different translocation groups. We show that a high number of novel splice loci is associated with adverse survival and an ultra-high risk group. The enumeration of patterns of alternative splicing has the potential to refine MM classification and to aid in the risk stratification of patients.
RNA 正常剪接模式的破坏是许多疾病发病机制的一个主要因素。越来越多的研究表明,剪接模式对癌症的进展有很强的影响。多发性骨髓瘤是一种分子异质性疾病,其特征是存在关键易位、基因表达谱和突变,但 MM 的剪接模式在很大程度上仍未得到探索。我们采用多方面的方法来确定 MM 中可变剪接的程度和影响。我们研究了剪接体成分 SF3B1,其热点突变(K700E 和 K666T/Q)导致其他癌症中可变剪接的增加。与 SF3B1 突变和野生型样本相比,我们发现了许多差异剪接的基因,包括 MZB1、DYNLL1、TMEM14C 和与剪接相关的基因 DHX9、CLASRP 和 SNRPE。我们发现异常剪接的作用更为广泛,在不同的易位组中,新的剪接变体的程度有明显的差异。我们表明,大量的新剪接位点与不良生存和超高风险组相关。可变剪接模式的列举有可能使 MM 的分类更加精确,并有助于对患者进行风险分层。