Variant snRNPs: New players within the spliceosome system.
Variant snRNPs: New players within the spliceosome system.
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变体SNRNP：剪接系统中的新玩家。
DOI：
10.1080/15476286.2017.1373238
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发表时间：
2018-01-02
期刊：
影响因子：
4.1
通讯作者：
O'Reilly D
中科院分区：
文献类型：
--
作者：
Vazquez-Arango P;O'Reilly D
现在越来越多的证据表明，除了它们在剪接中的一般作用外，核心剪接机制的组分具有广泛的调节潜力。特别是，最近的证据表明，这些因素的失调导致最高程度的选择性剪接的变化相比，经典的剪接调节器的失调。这种对剪接的一般抑制的缺乏与在与靶向核心剪接体组分的特异性突变相关的不同疾病病理中观察到的差异剪接效应产生共鸣。在这篇综述中，我们将总结什么是目前已知的核心spliceosomal U-snRNP复合物的干扰组织发育和人类疾病的参与，并认为存在一种补偿机制，使细胞能够科普激烈的干扰核心剪接组件。该系统通过变体（v）U-snRNP的差异表达维持剪接体snRNP的正确平衡。
Much evidence is now accumulating that, in addition to their general role in splicing, the components of the core splicing machinery have extensive regulatory potential. In particular, recent evidence has demonstrated that de-regulation of these factors cause the highest extent of alternative splicing changes compared to de-regulation of the classical splicing regulators. This lack of a general inhibition of splicing resonates the differential splicing effects observed in different disease pathologies associated with specific mutations targeting core spliceosomal components. In this review we will summarize what is currently known regarding the involvement of core spliceosomal U-snRNP complexes in perturbed tissue development and human diseases and argue for the existence of a compensatory mechanism enabling cells to cope with drastic perturbations in core splicing components. This system maintains the correct balance of spliceosomal snRNPs through differential expression of variant (v)U-snRNPs.
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