Article
Lamin B1 overexpression increases nuclear rigidity in autosomal dominant leukodystrophy fibroblasts.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology - 1 Sept 2014
Ferrera Denise, Canale Claudio, Marotta Roberto, Mazzaro Nadia, Gritti Marta, Mazzanti Michele, Capellari Sabina, Cortelli Pietro, Gasparini Laura
Abstract excerpt
The architecture and structural mechanics of the cell nucleus are defined by the nuclear lamina, which is formed by A- and B-type lamins. Recently, gene duplication and protein overexpression of lamin B1 (LB1) have been reported in pedigrees with autosomal dominant leukodystrophy (ADLD). However, how the overexpression of LB1 affects nuclear mechanics and function and how it may result in pathology remain...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.