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Novel roles for A-type lamins in telomere biology and the DNA damage response pathway.

The EMBO journal (2009-07-25)
Ignacio Gonzalez-Suarez, Abena B Redwood, Stephanie M Perkins, Bart Vermolen, Daniel Lichtensztejin, David A Grotsky, Lucia Morgado-Palacin, Eric J Gapud, Barry P Sleckman, Teresa Sullivan, Julien Sage, Colin L Stewart, Sabine Mai, Susana Gonzalo

A-type lamins are intermediate filament proteins that provide a scaffold for protein complexes regulating nuclear structure and function. Mutations in the LMNA gene are linked to a variety of degenerative disorders termed laminopathies, whereas changes in the expression of lamins are associated with tumourigenesis. The molecular pathways affected by alterations of A-type lamins and how they contribute to disease are poorly understood. Here, we show that A-type lamins have a key role in the maintenance of telomere structure, length and function, and in the stabilization of 53BP1, a component of the DNA damage response (DDR) pathway. Loss of A-type lamins alters the nuclear distribution of telomeres and results in telomere shortening, defects in telomeric heterochromatin, and increased genomic instability. In addition, A-type lamins are necessary for the processing of dysfunctional telomeres by non-homologous end joining, putatively through stabilization of 53BP1. This study shows new functions for A-type lamins in the maintenance of genomic integrity, and suggests that alterations of telomere biology and defects in DDR contribute to the pathogenesis of lamin-related diseases.

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