Department of Cell Biology, Lerner Research Institute, Cleveland Clinic, Cleveland, OH, USA.
Wiley Interdiscip Rev RNA. 2012 Jan-Feb;3(1):104-21. doi: 10.1002/wrna.107. Epub 2011 Aug 17.
RNA processing is important for generating protein diversity and modulating levels of protein expression. The CUG-BP, Elav-like family (CELF) of RNA-binding proteins regulate several steps of RNA processing in the nucleus and cytoplasm, including pre-mRNA alternative splicing, C to U RNA editing, deadenylation, mRNA decay, and translation. In vivo, CELF proteins have been shown to play roles in gametogenesis and early embryonic development, heart and skeletal muscle function, and neurosynaptic transmission. Dysregulation of CELF-mediated programs has been implicated in the pathogenesis of human diseases affecting the heart, skeletal muscles, and nervous system.
RNA 加工对于产生蛋白质多样性和调节蛋白质表达水平非常重要。CUG-BP、Elav 样家族(CELF)的 RNA 结合蛋白调节细胞核和细胞质中 RNA 加工的几个步骤,包括前体 mRNA 的可变剪接、C 到 U RNA 编辑、脱腺苷酸化、mRNA 衰变和翻译。在体内,CELF 蛋白已被证明在配子发生和早期胚胎发育、心脏和骨骼肌功能以及神经突触传递中发挥作用。CELF 介导的程序失调与影响心脏、骨骼肌和神经系统的人类疾病的发病机制有关。