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Biochem Soc Trans. 2008 Dec;36(Pt 6):1378-83. doi: 10.1042/BST0361378.

Exploring the effects of a dysfunctional nuclear matrix.

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  • 1Laboratory of Nuclear and Genomic Health, Centre for Cell and Chromosome Biology, Brunel University, Uxbridge, UK.

Abstract

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.

PMID:
19021559
[PubMed - indexed for MEDLINE]
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