Exploring the effects of a dysfunctional nuclear matrix.
The nuclear matrix has remained a contentious structure for decades; many believe that it is an artefact of harsh non-physiological procedures. However, its visualization using milder experimental techniques is leading to its general acceptance by the scientific community. It is a permanent network of core filaments underlying thicker fibres which is proposed to be a platform for numerous important nuclear activities such as transcription and DNA repair. Interestingly, A- and B-type lamin proteins and emerin are components of this nuclear structure; however, they are often referred to only as nuclear envelope proteins. The present mini-review intends to provide an overview of the nuclear matrix, mentioning both its constituents and functional significance. The impact of disease-causing mutations in both emerin and lamin proteins on the structure's ability to regulate and mediate nuclear processes is then discussed.
Elcock
Lauren S
LS
Laboratory of Nuclear and Genomic Health, Centre for Cell and Chromosome Biology, Brunel University, Uxbridge, UK.
Bridger
Joanna M
JM
D000818:Animals; D002875:Chromosomes / Q000378:metabolism; D004247:DNA / Q000378:metabolism; D016678:Genome / Q000235:genetics; D006801:Humans; D015530:Nuclear Matrix / Q000378:metabolism / Q000473:pathology*; D034521:Nuclear Matrix-Associated Proteins / Q000378:metabolism
D034521:Nuclear Matrix-Associated Proteins; D004247:DNA