TAILIEUCHUNG - Báo cáo khoa hoc : TDP-43: new aspects of autoregulation mechanisms in RNA binding proteins and their connection with human disease

The maintenance of correct protein homeostasis (‘proteostasis’) is an essen-tial activity of mammalian cells to preserve their vital properties and func-tions. Because of its importance, correct proteostasis is achieved by the cell in several ways and at several levels of each gene expression pathway. | IFEBS Journal MINIREVIEW TDP-43 new aspects of autoregulation mechanisms in RNA binding proteins and their connection with human disease Emanuele Buratti and Francisco E. Baralle InternationalCentre for Genetic Engineering and Biotechnology ICGEB Trieste Italy Keywords alternative splicing autoregulation hnRNP mRNA stability NMD SR proteins TDP-43 Correspondence F. E. Baralle Padriciano 99 34012 Trieste Italy Fax 39 040 375 7361 Tel 39 040 375 7337 E-mail baralle@ Received 4 February 2011 revised 8 April 2011 accepted 28 April 2011 doi The maintenance of correct protein homeostasis proteostasis is an essential activity of mammalian cells to preserve their vital properties and functions. Because of its importance correct proteostasis is achieved by the cell in several ways and at several levels of each gene expression pathway. In many cases mRNA-autoregulatory pathways based on a variety of feedback mechanisms have been observed to play a major role in keeping their concentration under control. This is especially true for RNA binding proteins because of their potential ability to bind their own pre-mRNA molecules and in particular for two subsets of nuclear factors that are commonly referred to as heterogeneous ribonucleoproteins and serine-arginine-rich proteins. Regarding the mechanism nonsense-mediated RNA degradation triggered by alternative splicing of their own messenger RNA is a very common autoregulation pathway to maintain constant expression levels within the cellular environment. Recently however alternative mechanisms other than nonsense-mediated decay have also been described to play a role for other RNA binding protein factors serine-arginine-rich splicing factor 1 SRSF1 and transactive response DNA binding protein 43 kDa TDP-43 . The aim of this minireview will be to discuss these old and new autoregulatory processes and their implication in disease development. Introduction With the term proteostasis .

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