Quality control of gene expression in the nucleus.
Proteins are responsible for most cellular and extra-cellular functions. If altered, proteins can loose their normal activity and/or gain new properties. Either way the consequences may be deleterious for the cell and lead to disease at the organism level. Not surprisingly, eukaryotes have evolved mechanisms to recognize abnormal messenger RNAs and prevent them from producing faulty proteins. Protein-encoding genes are transcribed in the nucleus by RNA polymerase II as precursor RNAs that undergo extensive processing before being translocated to the cytoplasm for translation by the ribosomes. This spatial and temporal separation between RNA and protein synthesis offers an immense opportunity for control and regulation. Here we review recent studies that are beginning to unravel how the coupling between transcription, processing and transport of mRNAs contributes to control the quality of gene expression in the nucleus.
Custódio
N
N
Institute of Histology and Embryology, Faculty of Medicine, University of Lisbon, Lisbon, Portugal.
Carmo-Fonseca
M
M
D002467:Cell Nucleus / Q000378:metabolism*; D005786:Gene Expression Regulation / Q000502:physiology*; D006801:Humans; D008954:Models, Biological; D009223:Myotonic Dystrophy / Q000378:metabolism; D011786:Quality Control; D012323:RNA Processing, Post-Transcriptional / Q000502:physiology*; D012326:RNA Splicing; D012333:RNA, Messenger / Q000378:metabolism*; D014158:Transcription, Genetic; D017086:beta-Thalassemia / Q000378:metabolism
D012333:RNA, Messenger